What must happen before symptoms of carcinoid syndrome manifest?
Answer
Carcinoid syndrome typically doesn't arise unless the tumor has metastasized to the liver/has seeded in the hepatic vein. This is because serotonin secreted by intestinal tumors will simply be degraded to 5-HIAA by liver MAO upon first-pass metabolism
Card 2
Why doesn’t a patient with carcinoid syndrome get left-sided valvular lesions?
Answer
The lung contains monoamine oxidase (MAO) which metabolizes the serotonin before it can return to the left heart
Card 3
What is carcinoid heart disease? Specifically how are the valves involved?
Answer
A complication of carcinoid syndrome that results in a right-sided valvular fibrosis, caused by serotonin-induced fibrosis (collagen deposition) of these valves. Triscuspid regurgitation and pulmonary valve stenosis are often seen.
Card 4
What is the wall involvement in Ulcerative Colitis vs Crohn Disease?
Answer
UC: mucosal and submucosal ulcers
CD: full-thickness inflammation, knife-like fissures and skip lesions
This is why CD and not UC can present with fistulas
Card 5
What are the anatomical differences between the location of ulcerative colitis vs Crohn disease?
Answer
UC: begins in the rectum, can ascend proximally as far as the cecum. Involvement is continuous and fully circumferential
CD: Anywhere along the GI tract; "skip lesions". Most common site is the terminal ileum. Rectum is the least common.
Card 6
What are the key histological findings of Ulcerative Colitis and Crohn Disease?
What disease presents with a "lead pipe" sign on imaging? What causes this?
Answer
Ulcerative colitis. Loss of haustra and development of pseudopolyps creates characteristic lead pipe image (on right)
Card 8
What disease presents with a "string-sign" on imaging? What causes this appearance?
Answer
Crohn disease. String-sign caused by colonic strictures.
Card 9
What are two high yield complications associated with UC?
Answer
Toxic megacolon (inflammation damages muscularis propria) and
carcinoma (risk is based on extent of colonic involvement and duration of disease)
*Also, as menioned in another card, primary sclerosing cholangitis
Card 10
What three diseases are positive for p-ANCA?
Answer
Microscopic polyangitis
Churg-Strauss (eosinophilic granulomatosis with polyangiitis)
and ulcerative colitis
Card 11
In relation to IBD: is smoking a risk factor for both types?
Answer
Smoking is a risk factor for developing Crohn disease.
However, it is protective against the development of ulcerative colitis.
Card 12
What are the symptoms of UC vs CD (pain localization, stool quality)?
Answer
Ulcerative colitis: LLQ pain (rectum) with bloody diarrhea.
Crohn Disease: RLQ pain (ileum) with non-bloody diarrhea
Card 13
The development of "cobblestone mucosa" is characteristic of which form of IBD? What is the pathophysiology behind its development?
Answer
Crohn Disease; cobblestone mucosa is caused by an aberrant healing process
Card 14
What are the extraintestinal associations of Ulcerative Colitis and Crohn Disease?
Answer
Ulcerative Colitis:
primary sclerosing cholangitis (suspect when patient presents with history of IBD and symptoms consistant with cholecystitis)
p-ANCA
Pyoderma gangrenosum
Crohn Disease:
arthritis (ankylosing spondylitis, sacroilitis, migratory polyarthritis),
erythema nodosum,
uveitis
*Recent thought is that you're gonna see any of these things in either disease.
Memory Tip:
UC - all the "p" symptoms
Crohn - imagine an old crone with red eyes and red bumpy skin (walking on cobblestones)
UC
Crohn
Card 15
Which type of IBD increases the risk of calcium oxalate nephrolithiasis?
Answer
Crohn Disease
Card 16
What syndrome is Hirschsprung disease associated with?
Answer
Down syndrome
*Hirschprung disease is a form of megacolon caused by failure of the colonic ganglion cells to develop. Affected areas cannot relax in order to pass stool.
Card 17
Why does Hirschsprung disease occur?
Answer
Congenital failure of ganglion cells (derived from neural crest) to descend into the myenteric and submucosal plexus
Card 18
What type of biopsy is needed to prove that the patient has Hirschprung's disease?
Answer
Rectal suction biopsy - suction is required to take a deep enough biopsy to see ganglion cells (or lack thereof) beneath mucosa
Card 19
Where is the most common location of a colonic diverticulum? What anatomical feature assists its development?
Answer
Sigmoid colon
Typically arises where the vasa recta traverse the muscularis propria (weak point in the colonic wall)
Card 20
What three high-yield complications are associated with colonic diverticula?
Answer
Rectal bleeding (hematochezia)
Diverticulitis - due to obstructing fecal material (appendicitis-like symptoms, but on the left)
Fistula - inflamed diverticula ruptures and attaches to a local structure. A colovesicular fistula can cause air or stool to be present in the urine.
Card 21
What is the most common cause of ischemic colitis?
Answer
Atherosclerosis of the SMA
Card 22
What are the 2 most common types of colonic polyp? Which might progress to cancer?
Answer
1) Hyperplastic ("serrated") polyp (no malignant potential)
Not to be confused with "sessile serrated" polyps (pre-malignant), in which neoplasia extends to crypts
2) Adenomatous polyp (pre-malignant)
Card 23
Describe the molecular progression from normal colonic mucosa to carcinoma:
Answer
1) APC mutation (both copies) → risk
2) K-ras mutation l → Polyp
3) p53 (or DCC) mutation & ↑ COX expression → Carcinoma
*This sequence is extremely high yield for examination purposes
Card 24
Why does taking aspirin confer protection against the development of colon cancer?
Answer
It inhibits COX, slowing the molecular mechanisms necessary for transition from adenomatous polyp to carcinoma
Remember, the adenoma-carcinoma sequence is characterized by the following mutations:
Loss of both APC genes: increased risk
KRAS mutation: polyp formation
Loss of p53 (or DCC, or both) and upregulation of COX: malignant transformation
Card 25
What hereditary disease is characterized by an inherited mutation in one copy of the APC gene?
Answer
Familial Adenomatous Polyposis - inherited APC mutation (chromosome 5)
Thousands of polyps develop in colon - high risk for malignant transformation
Card 26
What is Turcot syndrome?
Answer
FAP with CNS tumors (medulloblastoma and glial tumors)
Memory Tip:
Sounds like "twerk-it," which gives you brain damage
Card 27
What is Peutz-Jegher's syndrome? Which 4 cancers are these patients at higher risk for?
Answer
A syndrome characterized by:
hamartomatous (benign) polyps throughout the GI tract,
freckle-like spots on lips/oral mucosa, hands/feet, and genital skin
Increased risk for colorectal, breast, gynecological, and pancreatic cancers.
Card 28
What are the 3 types of cancer that patients with HNPCC are at risk for?
Answer
Colorectal, ovarian and endometrial carcinoma.
*HNPCC (formerly known as "Lynch syndrome") is an inherited defect in non-homologous end joining DNA repair
Card 29
What is HNPCC? Inheritance pattern?
Answer
An inherited (autosomal dominant) condition characterized by a mutation in DNA mismatch repair enzymes.
*Note: most cases of Lynch are caused by mutations in MSH2 (2p22) or MLH1 (3p21)
Card 30
The right and left sides of the colon are associated with which cancer pathways?
Answer
Left: adenoma-carcinoma sequence (usually FAP)
Right: microsatellite instability (usually HNPCC) (cancers arise de novo, not from polyps)
Card 31
What tumor marker is associated with colorectal cancer? How is this marker utilized in screening?
Answer
CEA
This is a trick question - CEA is not useful for screening, only for measuring response to treatment and detecting recurrence.
Card 32
Risk for an adenomatous polyp to undergo malignant transformation is assessed by which three histological properties?
Answer
Polyp is > 2 cm in size
Possesses sessile growth (vs. pedunculated)
Posseses villous histology: villous projections (vs. tubular spaces)
Card 33
Left-sided colorectal carcinomas usually present with what four clinical features?
How does infection with Tropheryma whippelii result in tissue damage? Where are the most common sites of tissue damage?
Answer
Tissue damage is characterized by accumulation of macrophages loaded with Tropheryma whippelii organisms. Macrophages are only partially able to kill organisms - they accummulate in lysozomes where they stain positive for PAS
Most common site: small bowel lamina propria (however, tissue damage can be systemic)
Memory Tip:
"CAN of whipped cream" - whipple can cause Cardiac, Arthralgias, Neurological symptoms
Card 35
This histology slide is characteristic of what infectious pathology?
Though this slide is a standard H&E, what other stain can be used to visualize the causitive organism?
Answer
Whipple's Disease - characterized b foamy macrophages within lamina propria.
A PAS stain shows the organisms inside the macrophage lyososomes.
Card 36
Why is the rate of recurrence so high for pleiomorphic adenoma?
Answer
Small islands of tumor typically extend through the tumor capsule, which often leads to an incomplete resection.
Card 37
What structure does a mucoepidermoid carcinoma typically involve?
Answer
Facial nerve
*Obviously "the parotid gland" is a valid answer, but all the salivary gland tumors involve the parotid gland, basically. It's high yield to understand that facial nerve symptoms are indicative of carcinoma invasion
Card 38
GERD is a metaplastic process: what type of epithelium does the esophagus normally possess? What type of epithelium does it transofrm into?
Answer
Non-keratinizing squamous epithelium → non-cilitated columnar epithelium with goblet cells
Card 39
What is a sliding hiatal hernia?
Answer
A diaphragmatic hernia, wherein the stomach herniates through the diaphragm via the esophagus
Card 40
Sliding hiatal hernias present with what classic imaging sign?
Answer
"Hourglass sign" on abdominal x-ray
Card 41
Paraesophageal stomach hernias are associated with what physical exam finding? When it occurs in a neonate, what is a possible consequence?
Answer
Bowel sounds in the lower lung fields
Lung hypoplasia in a neonate
Card 42
What are 4 clinical features of GERD?
Answer
1) Heartburn
2) Asthma
3) Damage to tooth enamel
4) Ulceration with stricture
Card 43
Esophageal carcinoma typically presents early or late? What is the prognosis?
Answer
Presents late. Prognosis is poor.
By the time symptoms appear, the tumor has usually spread to submucosal lymphatic vessels. 5-year survival is less than 25%, compared to 80% in cases limited to the mucosa or submucosa.
Card 44
What three clinical symptoms are both adenocarcinoma and squamous cell carcinoma of the esophagus associated with? What two clinical symptoms are particular to squamous cell carcinoma?
Answer
Shared:
1) Dysphagia
2) Weight loss
3) Hematemesis
Specific to SCC:
1) Hoarse voice (recurrent laryngeal nerve involvement)
2) Cough (tracheal involvement)
Card 45
What are the 3 levels of severity of gastric wall irritation?
Answer
1) Superficial inflammation
2) Erosion (loss of epithelium)
3) Ulcer (loss of mucosal layer)
Card 46
What are the 4 important clinical features of chronic autoimmune gastritis?
Answer
1) Atrophy of mucosa with intestinal metaplasia
2) Achlorhydria with increased gastrin levels and antral G-cell hyperplasia
3) Megaloblastic anemia due to lack of IF
4) Increased risk for gastric adenocarcinoma
Card 47
Describe the histological changes one will see in chronic autoimmune gastritis-induced intestinal metaplasia:
Answer
Replacement of foveolar cells (non-mucous secreting columnar cells) with mucous-secreting goblet cells, particularly within crypts (normal epithelium is boxed, abnormal is circled)
Card 48
What 2 virulence factors does H. pylori produce?
Answer
Ureases and proteases which degrade the gastric mucosa
*Ureases increase the gastric pH, inhibiting secretion of secretin and promoting secretion of gastrin;
overall effect is increased stomach acidity
Card 49
What is the classic histologic feature of duodenal ulcers?
Answer
hypertrophy of the Brunner glands - secrete alkaline mucin
Card 50
Duodenal ulcers most commonly occur on the anterior wall of the duodenem. However, in some instances, they occur on the posterior wall. These ulcers are at risk for rupture, which can lead to what two complications?
Answer
1) Bleeding from the gastroduodenal artery
2) Acute pancreatitis
Card 51
Rupture of a gastric ulcer can lead to what complication?
Answer
Bleeding of the left gastric artery
Card 52
Are duodenal ulcers predisposed towards malignancy?
Answer
No, Duodenal ulcers have practically no malignant potential
Card 53
Is this ulcer benign or malignant? What morphological features inform your opinion?
Answer
This is a benign ulcer. It is small, punched-out (or sharply demarcated) surrounded by non-piled radiating folds of mucosa
Card 54
Is this ulcer benign or malignant? What morphological characteristic inform your opinion?
Answer
Malignant. This ulcer is large and irregular with heaped-up margins
Card 55
What causes duodenal atresia?
Answer
Failure of the duodenum to recanalize during development
Card 56
What causes Meckel diverticulum?
Answer
Failure of the vitelline duct (embryonic structure connecting bowel to umbilicus) to involute.
*Remember that a persistent connection between the duct and the umbilicus can result in appearance of meconium around the belly button
Card 57
What causes a volvulus?
Answer
Twisting of a portion of the bowel along its mesentery. This twisting interrupts blood supply causing ischemia
*It's helpful to note that areas of the intestine with redundant mesentary are most prone to volvulus formation. This specifically includes the sigmoid colon and the cecum
Card 58
Emboli in which two locations are typically associated with transmural infarction of the small bowel? What are some underlying causes of these thromboses?
Answer
Transmural infarction of the small bowel is most commonly caused by:
thrombosis/embolism of the superior mesenteric artery - associated with atrial fibrillation, vasculitides (polyarteritis nodosa), atherosclerotic disease of the SMA or abdominal aorta (obvi)
Thrombosis of the mesenteric vein - polycythemia vera
Card 59
What type of small bowel infarction does not involve an embolus or thrombus?
Answer
Mucosal infarction of the small bowel is caused by marked hypotension
Card 60
What are the three key clinical features of small bowel infarction?
What are the two common presenting symptoms of lactose intolerance?
Answer
Abdominal distension and diarrhea upon consumption of milk
Card 62
What are two ways in which patients can acquire a lactase deficiency?
Answer
1) Loss of lactase enzyme with age
2) Infection of the brush border (temporary)
Card 63
What is the general pathophysiology of celiac disease?
Answer
Immune-mediated damage of small bowel villi, due to gluten exposure
Card 64
What HLA types is the development of celiac associated with?
Answer
HLA DQ2 and DQ8
"I 8 at Dairy Queen and got Celiac 2!"
Card 65
What is the most pathogenic (antigenic) component of gluten? How is it processed and presented?
Answer
Gliadin
Gliadin is deaminated by tTG (tissue transglutaminase) and presented by MHCII
Card 66
Anatomically speaking, where is damage caused by Celiac most prominent?
Answer
In the duodenum: jejunum and ileum mostly uninvolved
*Note that this makes a lot of sense - gliadin will be most concentrated at the site where it's released from the stomach. It dilutes the further down you go.
Card 67
What is the cause of tropical sprue?
Answer
An unknown organism causes tropical sprue.
Though it's never been isolated, we have observed that tropical sprue responds well to antiboiotics, which is why we believe a microorganism is responsible.
Card 68
In what geographical region does tropical sprue occur?
Answer
Mainly seen in the Carribbean
Card 69
What is the pathophysiology of fat malabsorption in Whipple's disease?
Answer
Accumulation of organism-laden macrophages in the lamina propria of the small bowel impinge upon the lymphatic lacteals (that chylomicrons are normally be absorbed into)
Card 70
What is abetalipoproteinemia?
Answer
An inherited autosomal recessive deficiency of B-48 and B-100
*Note: besides fat malabsorption, histology of lamina propria should show accumulation of intracellular fatty acids
Card 71
What roles do B-48 and B-100 play in fat absorption?
Answer
B-48 is necessary for the formation of chylomicrons
B-100 is necessary for the formation of VLDL and LDL (and IDL too, technically - mediates particle adhesion to LDL receptors for all lipoprotein particles)
Card 72
What simple screen is highly suggestive of carcinoid tumor?
Answer
Detection of 5-HIAA (a metabolite of serotonin) in the urine
Card 73
What is the classic triad of carcinoid syndrome?
Answer
1) Bronchospasm
2) Diarrhea
3) Flushing of the skin
*another variant is "FDR" - flushing, diarrhea, and R-sided heart failure
Card 74
An acute episode of carcinoid syndrome can be triggered by what two factors?
Answer
1) EtOH consumption
2) Emotional stress
Card 75
What is the most common cause of appendicitis in children? Adults?
Answer
In children: lymphoid hyperplasia
In adults: fecalith
*Either way, the initiating event is blockage of the appendix, creating a nidus for infection
Card 76
Guarding and rebound tenderness are signs of what complication in the setting of potential appendicitis?
Answer
Rupture (these are symptoms of acute peritonitis)
Card 77
What is a common complication of appendicitis (other than rupture)?
Answer
Periappendiceal abscess
Card 78
Epidemiology of IBD (typical patient)?
Answer
1) Typically a young woman (30s)
2) Prevalent in the west, among Caucasians and eastern European Jews
Card 79
What is the pathophysiology behind stricture development in Crohn Disease?
Answer
Deep erosion induces formation of granulation tissue with a predominance of myofibroblasts, whose main function is to facilitate wound contraction
Card 80
What are 4 potential complications of Crohn Disease (besides stricture formation)?
Answer
1) malabsorption (nutritional deficiency)
2) calcium oxalate nephrolithiasis
3) fistula formation (full-thickness)
4) carcinoma (if colonic disease is present)
*Malabsorption, fistula, and carcinoma should be obvious if you know the pathophysiology.
Would also note that anemia of chronic disease is also a complication that we might see in someone with long-term Crohn's.
Remember calcium oxalate nephrolithiasis: CrOhN
Card 81
What are the two ganglionic layers of the intestine and where are they found?
Answer
Myenteric (Auerbach) plex - found in the muscularis propria
Meissner's plexus - found in the submucosa
Remember, the order of intestinal layers from lumen outwards is:
Mucosa
Submucosa
Muscularis propria
Serosa (Adventitia if outside the peritoneum)
Card 82
What two areas of the colon are most affected by Hirschprung's Disease?
Answer
The rectum
Sometimes the distal rectal colon
Card 83
What are the usual presenting symptoms of Hirschprung's disease (4)?
Answer
1) Failure to pass meconium
2) Empty rectal vault (on digital rectal exam)
3) Massive dilation of bowel proximal to obstruction (risk of rupture)
4) Rectal suction biopsy reveals lack of ganglion cells
Card 84
What is the treatment for Hirshsprung Disease?
Answer
Surgical resection of the affected bowel
Card 85
Is a colonic diverticulum a true or false diverticulum?
Answer
False - the mucosa and submucosa protrude through the muscularis propria.
What is the most common anatomical location of of ischemic damage to the colon?
Answer
The splenic flexure. This is a watershed area of the SMA.
*Rectal area would be the second most common
Card 88
How does ischemic colitis typically present (3 symptoms)?
Answer
With postprandial pain,
weight loss,
instances of bloody diarrhea.
Card 89
What is angiodysplasia? Where does it typically arise? What is the presenting symptoms?
Answer
An acquired malformation of mucosal and submucosal capillary beds.
Arises in the cecum and the right colon due to high wall tension.
Presents as hematochezia in an older adult.
Card 90
Anatomically, where do angiodysplasia and diverticula commonly arise?
Answer
Both caused by high wall stress
Diverticula: left side of the colon
Angiodysplasia: right side of the colon
Card 91
What is hereditary hemorrhagic telangiectasia?
Answer
An autosomal dominant disorder resulting in thin-walled blood vessels, typically in the naso/oropharynx through the GI tract. Can present with hematochezia.
Telangiectasias seen on lips:
Card 92
What is irritable bowel syndrome (IBS)?
Answer
Relapsing abdominal pain with bloating, flatulence, and change in bowel habits. Improves with defacation.
Card 93
What would you likely see on colonic biopsy of a patient with irritable bowel syndrome?
Answer
No histologic changes.
Card 94
Who is typically affected with irritable bowel syndrome?
Answer
Middle-aged women
Card 95
How are colonic polyps screened for?
Answer
Colonoscopy and testing for fecal occult blood
Card 96
What is Gardner syndrome?
Answer
FAP presenting with fibromatosis (non-neoplastic proliferation of fibroblasts in the retroperitoneum) and osteomas
Card 97
What is a juvenile polyp?
Answer
Hamartomatous polyp that arises in children - usually a solitary polyp that prolapses and bleeds.
Card 98
What is juvenile polyposis?
Answer
A disease characterized by multiple juvenile polyps in the stomach (14%) and colorectum (98%)
Card 99
Right-sided colorectal carcinomas typically present with what three clinical features?
How do we approach iron deficiency anemia in an older adult?
Answer
Colorectal carcinoma (right-sided) until proven otherwise.
Card 101
S. bovis endocarditis is associated with what malignant pathology?
Answer
Colonic carcinoma
Card 102
What two types of necrosis are seen in acute pancreatitis, and what specific tissues undergo these necrotic processes?
Answer
Liquifactive necrosis of the pancreas itself
Fat necrosis of peripancreatic fat
Card 103
Which serum markers can be used to detect pancreatitis? Which is more specific for pancreatic damage?
Answer
Serum lipase and amylase; lipase (e.g. salivary glands also produce amylase)
*Note, both peak at around 6 hours after onset, but since lipase has a much longer half life (amylase excreted renally), it hangs around for up to 2-3 days post
Card 104
What are the two major risk factors for the development of pancreatic carcinoma?
Answer
Smoking and chronic pancreatitis
Memory Tips:
A shot goes down quick like acute pancreatitis; smoking the pancreas to death takes longer
Card 105
What is the serum tumor marker for pancreatic adenocarcinoma?
Answer
CA 19-9
Memory Tip:
Like "911." Pancreatic diseases present late (retroperitoneal), so this cancer is particularly deadly.
Card 106
What are 6 high-yield clinical features of pancreatic carcinoma?
Answer
1) Epigastric abdominal pain and weight loss
2) Obstructive jaundice with pale stools and palpable gallbladder
3) Secondary DM
4) Pancreatitis
5) Migratory thrombophlebitis (Trousseau sign)
6) CA 19-9 tumor marker
Card 107
Where is the most common location of a pancreatic carcinoma? What specific clinical feature of pancreatic carcinoma is this location associated with?
Answer
The head of the pancreas; obstructive jaundice (blockage of biliary outlet) with pale stools and palpable gallbladder (Courvoisier sign)
Card 108
What are the two types of gallstones? Which one is most common?
Answer
Cholesterol (most common) and bilirubin
*If asked on an exam: bilirubin stones in an actively hemolyzing person are 20% cholesterol, 80% bilirubin
Card 109
What are the risk factors for developing cholesterol cholelithiasis (6)?
Answer
1) Age (4 F's: "fat, female, forty, fertile")
2) Estrogen (female sex, obesity, multiple pregnancies, and oral contraceptives)
3) Cirrhosis (may contribute to estrogen)
4) Actively taking fibrates (particularly clofibrate)
5) Native American ethnicity
6) Crohn disease
Card 110
Which type of gallstone is radiolucent and which is radiopaque?
Answer
Radiolucent: cholesterol;
Radiopaque: bilirubin
Card 111
What 2 pathologies increase the risk for developing bilirubin cholelithiasis?
What gallbladder pathology is associated with Rokitansky-Aschoff sinus? What is a Rokitansky-Aschoff sinus?
Answer
Chronic cholecystitis;
herniation of gallbladder mucosa into the muscular wall.
Card 113
What is a porcelain gallbladder? Why must it be removed?
Answer
Shrunken, hard gallbladder due to chronic inflammation/fibrosis/dystrophic calification - caused by chronic cholecystitis.
It is removed (cholecystectomy) to prevent progression to carcinoma (gallbladder adenocarcinoma)
Card 114
An elderly woman who presents with cholecystitis - what important pathology should be in your differential?
Answer
Gallbladder carcinoma - (an adenocarcinoma arising from the glandular epithelium of the gallbladder wall). Poor prognosis.
Card 115
Why does the urine become dark with extravascular hemolytic processes or ineffective erythropoiesis?
Answer
Dark urine is due to increase in urine urobilinogen
*Important: never due to unconjugated (indirect) bilirubin - UCB is not water soluble and therefore not excreted in urine
Card 116
Which pattern of liver enzymes elevation is seen in viral hepatitis?
Answer
ALT > AST (AST:ALT ratio is less than 1)
Card 117
Which two hepatitis viruses are transmitted via the fecal-oral route?
Answer
HAV and HEV (fEcAL)
*As a reminder, HAV is a picornavirus, HEV is considered a Hepevirus
Hepatitis Mnemonics:
vowels (HepA/E) are AcutE
consonants (HepB/C/D) are Chronic
HepB: Blood, Birthing, "Bonking"
HepC: more often chronic (> 80% develop chronic hepatitis; of those, 20% develop cirrhosis)
HepD: delta agent, defective virus (i.e. requires HBV co-infection)
HepE: endemic in equatorial regions, frequently epidemic
Card 118
Why is it dangerous for a pregnant woman to contract HEV?
Answer
It is associated with fulminant hepatitis (liver failure with massive liver necrosis) in pregnant women specifically
Hepatitis Mnemonics:
vowels (HepA/E) are AcutE
consonants (HepB/C/D) are Chronic
HepB: Blood, Birthing, "Bonking"
HepC: more often chronic (> 80% develop chronic hepatitis; of those, 20% develop cirrhosis)
HepD: delta agent, defective virus (i.e. requires HBV co-infection)
HepE: endemic in equatorial regions, frequently epidemic
Card 119
HDV is dependent on what other virus in order to form a capsid?
Answer
HBV
Card 120
What is more severe: a coinfection or a superinfection of HBV and HDV?
Answer
A superinfection is more severe
*"Superinfection" denotes that HBV was contracted first, and HDV was acquired at a later time
*Interesting note: HDV is the most fatal hepatitis virus, with a mortality rate of 20%. It is highly associated with IV drug use
Hepatitis Mnemonics:
vowels (HepA/E) are AcutE
consonants (HepB/C/D) are Chronic
HepB: Blood, Birthing, "Bonking"
HepC: more often chronic (> 80% develop chronic hepatitis; of those, 20% develop cirrhosis)
HepD: delta agent, defective virus (i.e. requires HBV co-infection)
HepE: endemic in equatorial regions, frequently epidemic
Card 121
What are 3 clinical signs of excess estrogen in a patient with cirrhosis?
Condensed, damaged cytokeratin filaments
They are classically seen in a cirrhotic liver - most commonly a patient with chronic alcohol use, but they might also be present in Wilson Disease, so pay attention to the history
Card 123
What pattern of liver enzyme elevation would be seen upon alcoholic liver damage?
Answer
AST > ALT (AST:ALT ratio greater than 1)
Card 124
In hemochromatosis, what mediates tissue damage?
Answer
Generation of free radicals via the fenton reaction
Card 125
What is the classic triad of hemochromatosis? What are some other clinical findings?
Answer
Cirrhosis (iron deposition in the liver)
secondary diabetes (iron deposition in the islets)
bronze skin (iron deposition in the skin)
Other findings can include
dilated cardiomyopathy,
cardiac arrhythmias (dilation can disrupt conductive fibers), and
gonadal dysfunction
Pathophysiology:
In decreasing order of severity, hemosiderin deposits in:
liver → pancreas → myocardium → pituitary gland
→ adrenal gland → thyroid/parathyroid glands
→ joints → skin
In other words, by the time you see "bronze skin," there's tons of iron everywhere
Card 126
What type of carcinoma do patients with hemochromatosis have an increased risk of developing?
Answer
Hepatocellular carcinoma
Card 127
What is the treatment for Wilson disease? What defect is Wilson disease associated with?
Answer
D-penicillamine (chelates copper).
Wilson disease is caused by an inherited autosomal recessive defect in the ATP7B gene on chromosome 13.
*Contrast this with the X-linked recessive inherited defect in the ATP7A gene that causes Menkes disease
Card 128
What autoantibody is typically present in cases of primary biliary cirrhosis?
Answer
Anti-mitochondrial antibody
*Note that primary biliary cirrhosis is an autoimmune disease targeting the intrahepatic bile duct, not the common bile duct.
Card 129
What is Reye syndrome? What specific organelle is implicated in its development?
Answer
Fulminant liver failure and encephalopathy in kids with viral illnessnes who take aspirin.
It is likely related to mitochondrial damage of hepatocytes
*Note that in Kawasaki disease, despite it's very "viral-looking" presentation, the treatment is aspirin. Be really sure that it actually is Kawasaki first.
Card 130
What are the three general risk factors for hepatocellular carcinoma?
Answer
chronic hepatitis (HBV, HCV)
cirrhosis
aflatoxins (derived from Aspergillus, induce p53 mutations)
*Note that zinc deficiency is highly associated with increased risk of cirrhosis development
Hepatitis Mnemonics:
vowels (HepA/E) are AcutE
consonants (HepB/C/D) are Chronic
HepB: Blood, Birthing, "Bonking"
HepC: more often chronic (> 80% develop chronic hepatitis; of those, 20% develop cirrhosis)
HepD: delta agent, defective virus (i.e. requires HBV co-infection)
HepE: endemic in equatorial regions, frequently epidemic
Card 131
What is the serum tumor marker for hepatocellular carcinoma?
Answer
alpha-fetoprotein (AFP)
Card 132
What biliary tract disease is associated with concentric "onion skin" bile duct fibrosis leading to alternating strictures and dilation with "beading" of intra- and extrahepatic bile ducts on contrast imaging?
Answer
Primary sclerosing cholangitis
Onion skinning:
Card 133
What is the most common type of gall stone in the west? How do these stones appear on imaging?
Answer
Cholesterol Stones (yellow). They do not show up on X-ray (radiolucent).
Card 134
What findings can be identified in this pathological specimen taken from the gallbladder? Do these findings inform a particular diagnosis?
Answer
Rokitansky-Aschoff Sinus; Chronic Cholecystitis.
Gall bladder mucosa descends into the smooth muscle of the gallbladder.
Card 135
What is gallstone ileus?
Answer
Obstruction of the small bowel by gallstone.
Typically a a fistula must form between the gallbladder and small bowel in order to allow passage of a sufficiently sized stone, which then gets lodged in the ileocecal junction.
An important radiographic finding is air in the biliary tract - evidence of fistulization between the biliary system and the GI tract.
Card 136
In cases of acute viral hepatitis, how are liver enzyme levels in the blood affected? Where specifically does liver inflammation occur?
Answer
ALT > AST (reveresed in alcoholic damage)
Inflammation involves lobules of the liver and portal tracts ("spotty necrosis"); characterized by apoptosis of hepatocytes.
Compare this to chronic hepatitis, in which portal tracts (PV) are preferentially fibrosed.
*First aid would say that viral hepatitis initially involves "Zone I"
Card 137
What underlying pathology is this liver specimen indicative of?
Answer
Swollen, distorted, eosinophilic cells are identifiable as apoptotic hepatocytes: this is acute hepatitis (likely viral)
Card 138
Which anatomical areas of the liver are primarily affected in cases of chronic hepatitis?
Answer
Primarily involves inflammation of the portal tracts, not the lobules
*Contrast this is acute hepatitis, where both the lobules and the portal tracts are inflamed
Card 139
What serologic markers will be present in an acutely active HBV infection?
Answer
HBsAg - first serologic marker to rise, signals infectivity
Anti-HBcAg IgM - useful during window period, when HBsAg is cleared, but anti-HBsAg IgG still undetectable
Card 140
What pathology is this histological specimen, taken from the liver, indicative of?
Answer
Normal hepatic parenchyma is disrupted by broad bands of fibrosis and regenerative nodules of hepatocytes. This is classic liver cirrhosis.
Card 141
What can be appreciated in this histological specimen, taken from the liver?
Answer
This is classic alcohol related fatty liver; is reversible
Card 142
What does this histology slide, taken from a liver specimen, show? What pathological process are these findings indicative of?
Answer
Here we see swelling of the hepatocytes accompanied by formation of mallory bodies (eosinophilic cellular inclusions made of damaged cytokeratin). Note also the presence of fat accumulation, pushing our diagnosis more towards alcoholic hepatitis.
Card 143
What is the pathophysiology of primary hemochromatosis?
Answer
Primary hemochromatosis is an inherited (autosomal recessive) defect in iron absorption. Specifically - a C282Y mutation in the HFE gene.
Defective transport of iron out of cells results in the accumulation of iron, which damages tissues via radical generation via the fenton reaction
*Note: hemochromatosis is associated with HLA-A3
Card 144
What pathology is depicted below?
Answer
These are iron deposits in a patient suffering from hemochromatosis.
If difficulty in distinguishing between iron and lipofuschin persists, a prussian blue stain can be used, which will turn iron, but not lipofuscin, blue.
Prussian Blue stain in Hemochromatosis: Iron = Blue
Card 145
What conclusions would you draw from this pathology specimen taken from the liver?
Answer
Depicted are accumulations of lipofuscin - peroxisomal accumulation of pigment breakdown products. This is totally clinically benign, and must not be confused with the deposits seen in hemochromatosis
Card 146
This histology specimen, taken from the liver, is characteristic of what pathology?
Answer
The image depicts calssic "onion-skinning" periductal fibrosis, which is typical of primary sclerosing cholangitis.
*It is valuable to remind ourselves that on contrast imaging, the unaffected regions of the liver in PSC will dilate, causing a "beaded" appearance
Card 147
What is an "annular pancreas"? What condition can it cause?
Answer
Annular pancreas is a developmental malformation in which the pancreas forms a ring around the duodenum; risk of duodenal obstruction
*Vomiting is usually nonbilious, but may be bilious if stricture is distal to the ampulla of Vater
Card 148
What are the two most common causes of acute pancreatitis?
Can you list some other less-common causes?
Answer
Alcohol, gallstones
Other causes include:
trauma
hypercalcemia
hyperlipidemia (HY: familial type I and IV, not type II)
drugs
scorpion stings
mumps (POMP mnemonic)
Posterior duodenal ulcer rupture
Card 149
What are the 5 key clinical features of acute pancreatitis?
Answer
1) Epigastric abdominal pain that radiates to the back
2) Nausea and vomiting
3) Periumbilical/flank hemorrhage (enzymes eat through soft tissue)
4) Elevated amylase/lipase
5) Hypocalcemia (calcium consumed during fat saponification)
Card 150
What are 5 major complications of acute pancreatitis?
How does a pancreatic pseudocyst clinically present?
Answer
Abdominal mass with persistently elevated serum amylase
*Note: rupture results in release of enzymes into the abdominal cavity and hemorrhage
Card 152
What bacteria can colonize a pancreatic abscess?
Answer
E. coli
Card 153
Chronic pancreatitis is defined as "fibrosis of the pancreatic parenchyma", and is often secondary to recurrent bouts of acute pancreatitis. What are the two most common non-idiopathic causes of chronic pancreatitis?
Answer
1) Alcoholism (in adults)
2) Cystic fibrosis (in kids)
Card 154
What are 5 key clinical features of chronic pancreatitis?
Answer
1) (As with acute pancreatitis) Epigastric abdominal pain that radiates to the back
2) Pancreatic insufficiency and all consequences thereof (steatorrhea, fat soluble vitamin deficiencies, B12 deficiency)
3) Secondary DM (destruction of islets)
4) Dystrophic calcification of pancreatic parenchyma (same idea as porcelain gallbladder)
5) Increased risk for pancreatic carcinoma
Card 155
How does the dystrophic calcification seen in chronic pancreatitis characteristically appear on imaging?
Answer
"Chain of lakes" pattern on contrast studies due to pancreatic duct dilitations
Card 156
Are amylase and lipase useful biomarkers for chronic pancreatitis?
Answer
No. These are not elevated in chronic pancreatitis.
Card 157
Secondary DM in the setting of pancreatic carcinoma is most associated with what anatomical location of the tumor?
Answer
Secondary DM is most associated with tumors that arise in the body or tail of the pancreas.
Card 158
What is the epidemiology of pancreatic carcinoma (what does a typical patient look like)?
Answer
PC is most commonly seen in the elderly (median age = 70)
Card 159
What is standard surgical treatment for pancreatic carcinoma?
Answer
En bloc removal of the head and neck of the pancreas, proximal duodenum, and gallbladder. This is known as a Whipple procedure.
In other words, a section of duodenum plus everything touching it (distal stomach, gallbladder + proximal biliary tract, pancreas head) is removed; everything else is reassembled, with the remaining stomach anastomosed distal to the other structures.
Vocab: "-ectomy" is to remove, "-ostomy" in this case is to join two tubes.
Card 160
What is the prognosis for pancreatic adenocarcinoma?
Answer
1-year surival is 10%
Card 161
What is biliary atresia and how does it present?
Answer
Failure to form/early destruction of extrahepatic biliary tree.
Presents with jaundice within first 2 months of life. Can progress to cirrhosis.
Card 162
What are three common biliary tract infections that predispose towards the formation of bilirubin cholelithiasis?
Answer
1) E. coli
2) Ascaris lumbricoides (common roundworm in developing countries with poor water sanitation)
3) Clonorchis sinensis (Chinese liver fluke, also predisposes towards cholangitis and cholangiocarcinoma)
Card 163
What are the 6 general complications of either cholesterol or biliary cholelithiasis?
Answer
Though mostly asymptomatic, cholelithiasis can lead to:
1) Biliary colic (painful peristalsis against obstructed stone)
2) Acute cholecystitis (stasis enables bacterial proliferation)
3) Chronic cholecystitis
4) Ascending cholangitis (obstruct common bile duct)
5) gallstone ileus (erodes through and obstructs small bowel, e.g. at ileocecal valve)
6) gallbladder cancer (repeated damage/healing → accumulate mutations)
Card 164
What are the symptoms of biliary colic? How can the condition evolve?
Answer
Waxing and waning RUQ pain due to contraction against a stone lodged in the cystic duct
Symptoms are relieved if the stone passes
Card 165
In acute cholecystitis, an impacted stone in the cystic duct leads to what triad of gallbladder findings?
Answer
1) Diliatation with pressure ischemia
2) E. coli bacterial overgrowth
3) Inflammation
Card 166
What are the presenting symptoms and clinical features of acute cholecystitis (4)?
Answer
1) RUQ pain radiating to the right scapula (irritates phrenic nerve, same dermatomes [C3-5] as suprascapular nerve)
2) Increased WBC count
3) Nausea/vomiting
4) Increased serum alkaline phosphatase (produced by biliary epithelium; released with necrosis)
Card 167
How does chronic cholecystitis typically present?
Answer
Vague post-prandial RUQ pain
Card 168
Quantitatively, how much serum bilirubin is needed to cause visible jaundice?
Answer
> 2.5 mg/dL
Card 169
Describe the normal pathway of bilirubin metabolism from bilirubin capture in the reticuloendothelial system through excretion:
Answer
1) RBCs are consumed by macrophages of the reticuloendothelial system
2) Protoporphyrin is converted to unconjugated bilirubin
3) Albumin carries UCB to the liver
4) Uridine glucuronyl transferase (UGT) conjugates the bilirubin
5) Conjugated bilirubin (CB) is transferred to the bile cannaliculi to form bile in the GB
6) Bilirubin is released into the small bowel
7) Intestinal flora convert CB to urobilinogen
8) Urobilinogen is oxidized to stercobilin (stool excretion) and urobilin (urine excretion)
Memory Tips:
"Uro-" implies "to be in urine"
"-gen" is the precursor to whatever's before it
"ster" sounds like a pirate saying "stool." Arrrrgh!!! The statin pirates are taking over.
Card 170
Extravascular hemolysis/ineffective erythropoiesis can cause jaundice by overwhelming the liver's mechanism to metabolize and excrete bilirubin. What lab findings and clinical features would you expect in such a patient?
Answer
Increased unconjugated bilirubin;
Urine will be dark due to increased urobilinogen (UCB not directly excreted), increased risk for bilirubin gallstones.
Card 171
What causes physiologic jaundice of the newborn?
Answer
Transiently low uridine glucoronyl transferase (UGT) levels in the newborn liver. This is a fairly common condition.
Card 172
What is the pathophysiology of the neurological deficits seen in untreated physiologic jaundice of the newborn? What is the treatment?
Answer
Unconjugated bilirubin is fat soluble, and deposits in the basal ganglia ("kernicterus").
Treatment is phototherapy - which solublizes (does not conjugate) UCB.
Card 173
What is the difference between Gilbert Syndrome and Crigler-Najjar syndrome (at a molecular level)?
Answer
Gilbert syndrome = low UGT activity
Crigler-Najjar = absent UGT activity
Memory Tip:
"N" for "no" and "Najjar"
Card 174
What is Gilbert syndrome? How does it present? What is the prognosis?
Answer
An inherited (autosomal recessive) deficit in UGT. Results in jaundice during physiologic stress (infection). It is clinically insignificant.
Card 175
What is the cause of Crigler-Najjar syndrome (and inheritence pattern)? What is the prognosis?
Answer
An inherited (autosomal recessive) lack of UGT.
Crigler-Najjar is uniformly fatal. Accumulated UCB causes kernicterus (accumulation of bilirubin in the basal ganglia).
Card 176
What four diseases/conditions present with only increased unconjugated bilirubin (UCB) upon laboratory testing?
Answer
1) Extravascular hemolytic processes (liver UGT overwhelmed)
2) Physiologic jaundice of the newborn
3) Gilbert syndrome
4) Crigler-Najjar syndrome
Card 177
What is Dubin-Johnson syndrome?
Answer
An inherited (autosomal recessive) deficiency of bilirubin canalicular transport protein (MRP2). This leads to an accumulation of conjugated bilirubin.
Card 178
What are the lab findings and symptoms of Dubin-Johnson syndrome?
Similar to Dubin-Johnson syndrome - a defect in CB excretion. However, the liver does not become pigmented. Clinical/lab findings still include jaundice and increased CB.
Card 180
A biliary tract obstruction will cause these three blood/urine lab findings:
Answer
1) Increased conjugated bilirubin (NOT unconjugated)
2) Decreased urine urobilinogen/urobilin (bile must be metabolized in the intestine by gut flora, then reabsorbed before urinary excretation) - you might see increased bilirubin in the urine though
3) Increased alkaline phosphatase (produced by biliary epithelium; released with necrosis)
Card 181
What bilirubin-related lab findings does one expect to see in cases of viral hepatitis?
Answer
1) Increase in UCB (liver is damaged, cells release contents including UCB and CB)
2) Increase in CB
3) Increase bilirubin in urine (dark urine).
*Important to distinguish that urine color in viral hapatitis is caused by soluble bilirubin, rather than urobilinogen, as one might see in a case of extravascular hemolysis.
Card 182
What serologic marker can be elevated during the window phase (time between intitial infection and typical clinical detection) of HBV infection?
Answer
Anti-HBcAg IgM
Card 183
What serologic markers will be present in a patient with a resolved case of HBV infection?
Answer
Anti-HBcAg IgG (the immune response has matured)
Anti-HBsAg IgG
Card 184
What defines a chronic hepatitis B infection? What serologic marker might be present?
Answer
Chronic HBV in defined as the continued detectable presence of HBsAg for > 6 months.
Anti-HBcAg IgG present (unlike HBsAg, won't distinguish chronic from resolution)
HBeAg (used by virus to replicate) or HBV DNA may be present
Hepatitis Mnemonics:
vowels (HepA/E) are AcutE
consonants (HepB/C/D) are Chronic
HepB: Blood, Birthing, "Bonking"
HepC: more often chronic (> 80% develop chronic hepatitis; of those, 20% develop cirrhosis)
HepD: delta agent, defective virus (i.e. requires HBV co-infection)
HepE: endemic in equatorial regions, frequently epidemic
Card 185
What serologic marker will be seen in a patient who has been immunized against HBV?
Answer
Anti-HBsAg IgG
Card 186
What compound is the key mediator of alcoholic hepatitis?
Answer
Acetaldehyde (an ethanol metabolite)
Card 187
What three clinical features does Reye syndrome typically present with?
Answer
hypoglycemia (ASA metabolites inhibit mitochondrial enzymes → decreased β-oxidation)
elevated liver enzymes
nausea/vomiting
*Note that some sources state that Reye syndrome induces intracranial swelling - keep in mind that this can induce a cushing reflex: increased BP, irregular breathing, bradycardia
Card 188
Besides hepatitis viruses, what two other viruses can cause acute hepatitis?
Answer
EBV and CMV
Reminder of hepatitis mnemonics:
vowels (HepA/E) are AcutE
consonants (HepB/C/D) are Chronic
HepB: Blood, Birthing, "Bonking"
HepC: more often chronic (> 80% develop chronic hepatitis; of those, 20% develop cirrhosis)
HepD: delta agent, defective virus (i.e. requires HBV co-infection)
HepE: endemic in equatorial regions, frequently epidemic
Card 189
Hepatic cirrhosis is mediated by what cytokine, secreted from which cell, residing where?
Answer
TGF-β secreted from stellate cells which lie beneath the endothelial layer lining the sinusoids
*Nice little immuno correlate: TGF-β is a big inducer of fibroblasts, which makes a lot of sense, since hepatic cirrhosis is highly fibrotic
Card 190
What are the four clinical sequelae of portal hypertension?
Answer
1) Ascites
2) Congestive splenomegaly/hypersplenism
3) Portosystemic shunts (various varices)
4) Hepatorenal syndrome (renal failure)
"Hypertension in a portly CHAP"
Card 191
Decreased detoxification (of ammonia) in the setting of liver cirrhosis can lead to what 3 clinical findings?
What is primary biliary cirrhosis? What type of patient is it associated with?
Answer
Autoimmune granulomatous destruction of the intrahepatic bile ducts;
women, average age 40, prexisting autoimmune condition
Card 198
What serum marker, seen in certain vasculitides and UC, is often positive in cases of primary sclerosing cholangitis?
Answer
p-ANCA
Ulcerative Colitis associations:
(reminder: they all start with "p")
Card 199
Primary sclerosing cholangitis increases risk for what malignancy?
Answer
Cholangiocarcinoma
Card 200
What is hepatic adenoma associated with? What complication can it cause? What is the treatment?
Answer
These are benign tumors that are seemingly induced by estrogen. They can rupture and cause intraperitoneal bleeding. They are commonly associated with oral contraceptive use, and treatment is cessation
Card 201
Hepatocellular carcinoma increases risk for what syndrome?
Answer
Budd-Chiari syndrome
Card 202
What is the most common liver malignancy?
Answer
Trick question: metastasis from a distant tumor
Card 203
How do mets to the liver typically present?
Answer
Hepatomegaly with a nodular free edge of the liver
Card 204
What is the most common congenital renal anomaly? Decribe the anatomy of this anomaly:
Answer
Horseshoe kidney.
Kidneys connected at the lower pole. Become abnormally located in the lower abdomen, because they get caught on the inferior mesenteric artery root.
Card 205
Bilateral renal agenesis causes what pathological sequence? Describe:
Answer
Lack of kidneys causes oligohydramnios (failure to produce amniotic fluid) with
lung hypoplasia,
flat face with low-set ears, and
developmental defects in the extremities.
This is called the Potter sequence.
Memory Tip:
Voldemort from the Harry Potter series had a messed up nose.
Card 206
How can you clinically distinguish between dysplastic kidney and polycystic kidney disease?
Answer
Dysplastic kidney (cystic congenital malformation of the kidney parenchyma) is typically unilateral.
PKD is an inhereted condition that will present with bilateral kidney involvement.
Card 207
What are the two presentations of polycystic kidney disease?
What should you suspect in a patient with a family history of renal disease and death occuring because of renal disease or brain hemorrhage? What 3 extrarenal symptoms do you expect? What is the underlying mutation?
Answer
ADPKD, which can be associated with berry aneurysms, hepatic cysts, and mitral valve prolapse.
Due to mutations in APKD1 (chromosome 16) and 2 (chromosome 4)
Card 209
If a newborn presents with Potter sequence and portal hypertension, what should you suspect?
Answer
ARPKD; associated with congenital hepatic fibrosis and hepatic cysts
Card 210
Medullary cystic kidney disease has what mode of inheritence? What does it cause?
Answer
Autosomal dominant defect leading to cysts in the medullary collecting ducts. Parenchymal fibrosis will lead to shrunken kidneys and worsening renal failure.
Card 211
What leads to prerenal azotemia? What leads to postrenal azotemia?
Answer
Prerenal: decreased blood flow to the kidneys (CHF, renal artery stenosis)
Postrenal: obstruction of urinary tract downstream from the kidney
Card 212
How do lab values (BUN:Cr, FENa, urine osm) change between early and late-stage postrenal azotemia?
Answer
Early stage:
serum BUN:Cr ratio >15 (obstructive backpressure increases BUN reabsorption),
tubular function remains intact FENa <1%
and urine osm >500 mOsm/kg - looks like prerenal azotemia
Long-standing obstruction:
serum BUN:Cr ratio <15 (functional tubules needed to reabs BUN),
decreased reabsorption of Na (FENa > 2%) and
inability to concentrate urine (urine osm < 500 mOsm/kg)
Card 213
What is the most common cause of acute renal failure (intrarenal azotemia)?
Answer
Acute tubular necrosis
as opposed to acute interstitial nephritis (drugs) or renal papillary necrosis (mostly diseases, i.e. rarer than environmental exposures)
Card 214
Clinical features of acute tubular necrosis (3)?
Urine findings?
BUN and Cr?
Electrolyte abnormalities?
Answer
1) Brown, granular casts seen in the urine (necrotic cells sloughing off)
2) Elevated BUN and creatinine (poor excretion)
3) Hyperkalemia (decreased renal excretion) with metabolic acidosis.
Card 215
What are the common causes of nephrotoxic-acute tubular necrosis (1 physiologic cause, 6 nephrotoxic agents)?
Answer
1) Ischemia
2) Nephrotoxic agents
- ethylene glycol (oxalate crystals in urine),
- contrast dyes
- urate (tumor lysis syndrome)
- aminoglycosides,
- heavy metals (lead),
- myoglobin
Memory Tips:
ethylene glycol - antifreeze, the green ooze stuff
contrast dye - the black/white monitor contrast symbol in the back
urate - crab, i.e. cancer, causes TLS when it dies quickly
aminoglycosides - the evil green bacteria blob, also recall the nephrotoxic Sketchy symbol in aminoglycosides
heavy metals - get it? so heavy the crab is tilted (unlike the DCM preggo lady)
myoglobin - muscle
Card 216
What are the causes of acute interstital nephritis?
Answer
NSAIDs, penicillin, diuretics (AID is an acute hypersensitivity reaction involving the interstitium and tubules)
Card 217
What are the clinical features of acute interstitial nephritis? What may AIN progress to?
Answer
Oliguria, fever, and rash (e.g. drug-induced T1/T4 HSR). Eosinophils in the urine are pathognomonic.
Can progress to renal papillary necrosis.
Card 218
What are the 4 common causes of renal papillary necrosis?
What protein imbalances/blood abnormalities can nephrotic syndrome result in (what proteins are lost in urine) (4 substances)?
Answer
1) Hypoalbuminemia (pitting edema),
2) Hypogammaglobulinemia (increased risk of infection),
3) Hypercoagulable state (due to loss of antithrombin III),
4) Hyperlipidemia and hypercholesterolemia (may result in fatty casts in urine)
Card 220
Which nephrotic syndrome is associated with Hodgkin's lymphoma?
Answer
Minimal change disease (MCD). However, most cases are idiopathic.
The cytokines produced by Reed-Sternberg cells are what damage podocytes; hence, MCD responds to steroids
Card 221
What are the key clinical findings of minimal change disease (5)?
H&E appearance?
EM?
Immunofluorescence?
How is proteinuria characterized?
Response to steroids?
Answer
1) Normal glomeruli on H&E stain
2) Effacement of foot processes seen on EM
3) No immune complex deposits (clear immunofluorescence)
4) Selective proteinuria (loss of albumin but not immunoglobulin)
5) Excellent response to steroids (damage is T cell cytokine mediated)
Card 222
What is the most common cause of nephrotic syndrome in Hispanics and African Americans?
Answer
Focal segmental glomerulosclerosis (FSGS)
Card 223
What three conditions can FSGS be associated with?
Answer
1) HIV
2) Heroin use
3) Sickle Cell Disease
Card 224
What is the most common cause of nephrotic syndrome in caucasian adults?
Answer
Membranous nephropathy
*Reminder: it's associated with SLE
Card 225
What are 5 causes of membranous nephropathy?
Answer
1) Idiopathic (most common)
2) Hepatitis B or C
3) Solid tumors
4) SLE
5) Drugs (NSAIDs and penicillamine)
Memory Tips:
Idiot hippie - idiopathic, Hep B/C
Crab - solid tumor
Wolf face - SLE
Psychedelic background - drugs
Skewer - "spike" and dome
Card 226
3 lab features of membranous nephropathy?
H&E?
Immunofluoresence?
EM?
Answer
1) Thick glomerular basement membrane on H&E
2) Granular immunofluorescence due to immune complex deposition
3) Subepithelial deposits with 'spike and dome' appearance
Card 227
What nephrotic syndrome appears with a "tram track" apperance with thick glomerular basement membrane on H&E?
Answer
Membranoproliferative glomerulonephritis
Card 228
Where are the deposits in type I and II membranoproliferative glomerulonephritis? What underlying disease states is each type associated with?
Answer
Type I: subendothelial deposits; associated with HBV and HCV.
Type II: dense intramembranous deposits; associated with C3 nephritic factor (autoantibody that stabilizes the C3 convertase, leading to over-activation of complement and low levels of circulating C3)
Card 229
In what nephrotic syndrome will you see Kimmelstiel-Wilson nodules? Why?
Answer
Kimmelsteil-Wilson nodules represent sclerosis of the mesangium as induced by high glomerular filtration pressure.
Most commonly seen in diabetic nephropathy, since non-enzymatic glycosylation of the vascular basement membrane and subsequent hyaline arteriolosclerosis affects the glomerular efferent arteriole more than the afferent.
Card 230
What is the most commonly involved organ in systemic amyloidosis? What is the result?
Answer
The kidney; amyloid deposits in the mesangium, resulting in a nephrotic syndrome
Card 231
What are the 6 general principles/characteristics of nephritic syndrome?
How much protein?
Urine composition (gross)?
Symptoms?
Urinalysis?
Glomeruli on H&E?
Mechanism of disease?
Answer
1) Limited proteinuria (< 3.5 mg/day)
2) Oliguria and azotemia
3) Salt retention with periorbital edema and hypertension
4) RBC casts and dysmorphic RBCs in urine
5) Glomeruli are hypercellular and inflamed
6) Immune complex deposition activates complement; C5a attracts neutrophils which mediate damage
Card 232
4 clinical features of poststreptococcal glomerulonephritis:
Time of presentation?
Symptoms?
H&E?
EM?
Answer
1) Presents 2-3 weeks after infection with Group A Strep
2) Hematuria, oliguria, hypertension, periorbital edema
3) Hypercellular inflamed glomeruli on H&E
4) Granular subepithelial "humps" on immunofluorescence (immune complex mediated)
Card 233
What defines the nephritogenic strains of Streptococcus?
Answer
Possessing M protein
Card 234
What is the composition of the crescents in rapidly progressive glomerulonephritis (RPGN)? Where are the crescents located?
Answer
Crescents made of fibrin and macrophages accumulating in Bowman's space
Card 235
What 3 diseases can result in/progress to rapidly progressive glomerulonephritis (RPGN)?
Answer
Goodpasture syndrome
Wegener's granulomatosis (GPA)
Microscopic polyangitis
(PSGN can do it too!)
Card 236
What is the immunofluorescence pattern for Goodpasture syndrome-induced rapidly progressive glomerulonephritis (RPGN)?
Answer
Linear (anti-basement membrane antibody)
Card 237
What is the immunofluorescence pattern for wegener's granulomatosis- and microscopic polyangitis-induced rapidly progressive glomerulonephritis (RPGN)?
Answer
Negative immunofluorescence (pauci-immune);
Card 238
What pattern of immunofluorescence would be seen in a case of PSGN-induced rapidly progressive glomerulonephritis (RPGN)?
Answer
Granular - due to immune complex deposition
Card 239
How long does it take for rapidly progressive glomerulonephritis to progress to renal failure?
Answer
Weeks to months
Card 240
What is Alport syndrome? What are 3 key symptoms?
Answer
X-linked defect in type IV collagen
1) Thinning and splitting of the glomerular basement membrane, causing hematuria
2) Sensory hearing loss
3) Ocular disturbances
Card 241
What is IgA nephropathy (Berger disease)? How common is it?
Answer
IgA immune complex deposition in the mesangium of the glomeruli.
It is the most common nephropathy worldwide.
Card 242
What is the most common type of renal disease in SLE?
Answer
Diffuse proliferative glomerulonephritis (DPGN)
Card 243
3 risk factors for developing any kind of urinary tract infection?
Answer
1) Sexual intercourse
2) Urinary stasis
3) Catheters
Card 244
What 3 lab finding are indicative of cystits?
Urinalysis:
Dipstick:
Culture:
Answer
1) Urinalysis: cloudy urine with > 10 WBCs/high power field
2) Dipstick: positive leukocyte esterase (due to pyuria) and nitrates (bacteria produce nitrates)
3) Culture: greater than 100,000 CFU (gold standard)
Card 245
Which part of the kidney makes erythropoietin?
Answer
The renal peritubular interstitial cells (closely assoc w/ peritubular capillaries)
Card 246
Where does renal cell carcinoma arise from?
What is the classic triad?
What syndrome is it associated with?
Answer
It arises from the kidney tubules.
Triad: hematuria, palpable mass, and flank pain. Hematuria is the most common symptom.
It is associated w/ von-Hippel-Lindau syndrome or sporadic deletion of the VHL gene (chromosome 3)
Card 247
5 substances that can be ectopically secreted by renal cell carcinoma, and associated symtpoms:
Answer
EPO (leading to reactive polycythemia vera),
Renin (leading to HTN),
PTHrP (leading to hypercalcemia),
ACTH (leading to Cushing's),
Prolactin (decreased libido, galactorrhea)
Card 248
Does renal cell carcinoma present with a right or left sided varicocele?
Answer
Left sided varicocele - left spermatic vein drains into the renal vein, which can be invaded. The right spermatic vein drains diractly into the IVC, and is practically never involved.
Card 249
What is the most common variant of renal cell carcinoma?
Answer
Clear cell type
Card 250
What gene deletion is associated with renal cell carcinoma? How does this deletion induce tumorigenesis?
Answer
Loss of VHL tumor suppressor gene (3p)
Causes increased IGF-1 (promotes growth) and,
Increased HIF transcription factor, which increases VEGF and PDGF to promote angiogenesis
Card 251
Besides renal cell carcinoma, what other cancer are patients with VHL at risk for developing?
Answer
Hemangioblastoma of the cerebellum
Card 252
What is the most common malignant renal tumor in children?
Answer
Wilms tumor - average age is 3 years
Card 253
What is WAGR syndrome? Denys-Drash syndrome? Beckwith-Widemann syndrome? How are these entities genetically different?
Answer
WAGR syndrome: Wilm's tumor, Aniridia, Genital abnormalities and mental and motor Retardation.
- associated with deletion of the WT1 gene
Denys-Drash syndrome: Wilms tumor, progressive renal disease, male pseudohermaphroditism
- associated with WT1 mutations
Beckwith-Widemann syndrome: Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy and organomegaly (including tongue)
- Mutations in the WT2 cluster, particularly IGF-2
Desperate Memory Tips:
Denys-Drash: like "Denny dash," hurry to go to bathroom at Denny's, GU tract involved
Beckwith-Widemann: "Wide man" genes are size "2" and not "1"; so wide on one side of hyphen, like imbalanced muscles
Card 254
What is the cell composition of a Wilms tumor?
Answer
Blastema (immature kidney mesenchyme)
primitive glomeruli and tubules
Stromal cells
Card 255
What is the most common type of lower urinary tract cancer?
Answer
Urothelial (transitional cell) carcinoma
Card 256
What is the major risk factor for Urothelial (transitional cell) carcinoma? What are 3 other risk factors?
Answer
1) Cigarette smoke
2) Naphthylamine
3) Azo dyes
4) Long-term cyclophosphamide
Memory Tips:
NITROsamine - can eat something cooked w/ nitro fuel
naphTHYLamine - "filling" is a bladder problem (filling the stomach? no problem!)
Card 257
What are 3 risk factors for squamous cell carcinoma of the bladder?
An adenocarcinoma that arises from the urachal remant is present where on the bladder?
Answer
It is present at the dome of the bladder
Card 259
What kidney pathology?
Answer
Focal segmental glomerulosclerosis
Card 260
What kidney pathology does this histology suggest?
Answer
Membranous nephropathy - note the thick glomerular basement membranes
Card 261
What kidney pathology does this EM slide suggest?
Answer
Membranous nephropathy - subepithelial deposits on EM, 'spike and dome'
Card 262
What do you see in this histology specimen? What is the likely diagnosis?
Answer
Kimmelstein Wilson Nodules - diabetic nephropathy.
Card 263
Subepithelial humps on EM - diagnosis?
Answer
PSGN - subepithelial humps without spike & dome (i.e. not membranous nephropathy)
Card 264
What does this histology specimen show? Diagnosis?
Answer
Thyroidization of the kidney - chronic pyelonephritis
Card 265
Histology taken from a kidney tumor. Diagnosis?
Answer
Renal cell carcinoma, clear cell type
Card 266
What urogenital tract malignancy?
Answer
Urothelial (transitional cell) carcinoma - flat type
Card 267
What is azotemia?
Answer
Increased BUN and creatinine, often with oliguria
Card 268
In cases of prerenal azotemia, what do you expect the BUN:Cr ratio to be?
Answer
BUN:Cr ratio > 15 (15 is normal)
Card 269
Does tubular function remain intact in prerenal azotemia? What lab values inform your answer?
Answer
Tubular function remains intact in prerenal azotemia.
The fractional excretion of sodium (FENa) will be < 1%,
Urine osmolality will be > 500 mOsm/kg
Card 270
How is acute tubular necrosis treated?
Answer
With supportive dialysis - electrolyte imbalances can be fatal. Disease can and often will resolve.
Card 271
How long after recovery from acute tubular necrosis can oliguria persist?
Answer
2-3 weeks.
Card 272
What are 4 key clinical features of focal segmental glomerulosclerosis?
H&E?
EM?
Immunofluorescence?
Response to steroids?
Answer
1) Focal (some glomeruli) and segmental (involving only part of the glomerulus) sclerosis on H&E
2) Effacement of foot processes on EM
3) No immune complex deposits, negative IF
4) Poor response to steroids - often progresses to renal failure
Card 273
How well does membranous nephropathy respond to steroids?
Answer
Poorly
Card 274
How well does membranoproliferative glomerulonephritis respond to steroids?
Answer
Poorly
Card 275
What therapeutic can help to slow progression of hyperfiltration-induced damage in diabetic nephropathy?
Answer
ACE inhibitors
Card 276
What is a sequela of poststreptococcal glomerulonephritis in an adult?
Answer
25% of adult cases progress to rapidly progressive glomerulonephritis (RPGN)
Card 277
How does IgA nephropathy (Berger disease) present?
Answer
During childhood, as episodic gross or microscopic hematuria with RBC casts. Follows mucosal infections.
Card 278
What are the common symptoms of cystitis? What symptom is notably absent?
Answer
Dysuria, increased urinary frequency, suprapubic pain; fever is notably absent
Card 279
What are the 5 top causes of cystitis?
Answer
1) E. Coli (80% of cases)
2) Staph Saprophyticus (young, sexually active women, although E. Coli is still more common in these patients)
3) Klebsiella pneumoniae
4) Proteus mirabilis (alkaline urine with ammonia scent)
5) Enterococcus faecalis (more common than E. faecium)
Card 280
What is sterile pyuria and what pathogens does it suggest in the context of urethritis?
Answer
Sterile pyuria is the presence of pyuria (> 10 WBCs/hpf and (+)leukocyte esterase) with a negative urine culture.
Suggests urethritis due to Chlamydia or gonorrhea. In rare instances, can be urethritis due to TB.
Card 281
The presence of WBC casts in the urine is indicative of what pathology?
Answer
Pyelonephritis - cast in the shape of kidney tubules
Card 282
What are the 3 most common culprits of pyelonephritis?
Answer
1) E. Coli (90% of cases)
2) Enterococcus faecalis
3) Klebsiella
Card 283
What are two contributing factors to chronic pyelonephritis?
Answer
1) Vesicoureteral reflux (children)
2) Obstruction - BPH or cervical carcinoma (adults)
*Reminder: cervical cancer invades bladder to cause obstruction
Card 284
What is the classic pattern of damage seen in chronic pyelonephritis?
Answer
1) Cortical scarring with blunted calyces
2) Scarring at specifically the upper and lower poles is seen when chronic pyelonephritis is due to vesicoureteral reflux
Card 285
What is "thyroidization" of the kidney?
Answer
A consequence of chronic pyelonephritis, where eosin-filled atrophic tubules resemble thyroid follicles.
Waxy urinary casts are often associated with thyroidization.
Card 286
Most common type of kidney stone?
Answer
Calcium oxalate/calcium phosphate
Card 287
What is the most common cause of calcium oxalate nephrolithasis? How is it treated?
Answer
Idiopathic hypercalciuria; must exclude hypercalcemia and related causes, also Crohn's.
Treat with hydrochlorothiazide (calcium sparing diuretic)
Card 288
What two organisms tend to cause ammonium magnesium phosphate nephrolithiasis? Why these two?
Answer
Proteus spp. and Klebsiella; both are urease(+) organisms, which alkalinize the urine, causing precipitation of the stone.
Card 289
Staghorn calculi can be composed of what two materials?
1) Alkalinization of urine (potassium bicarb)
2) Hydration
3) Allopurinol (or febuxostat 2nd line for overproduction; Rx probenecid/high-dose ASA/pegloticase for underexcretion)
Card 294
Cystine nephrolithiasis forms in the context of what disease?
Answer
Cystinuria (a defect in the renal tubules that results in decreased reabsorption of cysteine)
Card 295
How can cystine kidney stones be treated (2)?
Answer
1) Hydration
2) Alkalinization of urine
Card 296
In renal failure, 4 substances that should be excreted by the kidney are not. What are the effects?
Further, 2 endocrine functions performed by the kidney are impaired. What are these?
Answer
1) Urea - leads to uremia - anorexia, pericarditis, asterixis, platelet dysfunction, etc.
2) Sodium - leads to water retention - HTN
3) Potassium - leads to hyperkalemia with metabolic acidosis
4) Phosphate - leads to hypocalcemia, secondary hyperparathyroidism, renal osteodystrophy
1) Low EPO production by renal peritubular interstitial cells - anemia
2) 1-a-hydroxylation of vitamin D by proximal renal tubule cells - exacerbates hypocalcemia
Card 297
What are the treatment options for end-stage kidney failure?
Answer
1) Dialysis
2) Kidney transplant
Card 298
What is an angiomyolipoma?
Answer
A hamartoma of the kidney composed of blood vessels, smooth muscle, and fat.
Common finding in patients with tuberous sclerosis
Card 299
In whom does renal cell carcinoma sporadically arise? Where? What is the single most important risk factor?
Answer
1) Older men (average age is 60)
2) Arises at upper pole of the kidney
3) Smoking is the major risk factor
Memory Tip:
RCC affects upper pole like how emphysema mainly affects the upper lung lobes; smoking is a risk factor for both
Card 300
How is renal cell carcinoma staged?
Answer
"TN" system:
T - based on size and involvement of renal vein (increased risk of hematogenous spread to lungs and bone)
N - spread to retroperitoneal lymph nodes
Card 301
How does Wilms tumor typically present (what vital sign is typically elevated)?
Answer
Large, unilateral flank mass with hematuria and hypertension (tumor secretes renin)
Card 302
What percentage of Wilms tumor cases arise sporadically?
Answer
90%
Card 303
In whom does urothelial (transitional cell) carcinoma typically present? How does it present?
Answer
1) Older males
2) Painless hematuria
Card 304
Describe the two pathways by which urothelial (transitional cell) carcinoma can arise:
Answer
1) Papillary pathway - develops as a low-grade papillary tumor that progresses to a high grade papillary tumor then invades. Not associated with early p53 mutations
2) Flat pathway - develops as a high grade flat tumor and then invades, associated with early loss of p53
Card 305
What is a "field defect" in the context of urothelial (transitional cell) carcinoma?
Answer
Refers to the fact that tumors are multifocal and often recur
Card 306
What urogenital tract malignancy?
Answer
Urothelial (transitional cell) carcinoma - papillary type
Card 307
Condyloma are typically due to which strains of HPV?
Answer
6,11
Card 308
What are the key low-risk and high-risk strains of HPV?
Answer
Low-risk: 6,11
High-risk: 16,18, 31, 33
Card 309
What is the histologic hallmark of HPV-induced condyloma?
Answer
Koilocytes (cells with enlarged nuclei and hyperchromasia)
Card 310
Lichen sclerosis is characterized by what changes to the vaginal skin?
Answer
1) Thinning of the epidermis
2) Fibrosis (sclerosis) of the dermis
Card 311
In whom does lichen sclerosis mostly present? What can it increase the risk for?
Answer
Postmenopausal women - increases risk of developing squamous cell carcinoma of the vulva
Card 312
Compare and contrast lichen sclerosis with lichen simplex chronicus (across 4 criteria):
Answer
1) Thinning of epidermis with fibrosis of dermis. Hyperplasia of vulvar squamous epithelium
2) Parchment-like vulvar skin. Leathery vulvar skin
3) Autoimmune etiology. Chronic irritation and scratching
4) Increased risk of SCC. No increased risk.
Card 313
What three conditions can present with leukoplakia of the vulvar skin?
1) Induced by infection with HPV 16 or 18
2) Arising from longstanding lichen sclerosis
Card 315
What are the risk factors for HPV exposure?
Answer
Multiple partners, early first age of intercourse.
Card 316
HPV-related vulvar carcinoma usually arises in women of what age?
Answer
Women of reproductive age. Contrast this to VC that arises from lichen sclerosis, where the average presenting age is >70.
Card 317
What is extramammary Paget disease and how does it present?
Answer
Malignant epithelial cells proliferating in the epidermis of the vulva.
Presents as an erythematous, pruritic, ulcerated vulvar lesion.
Card 318
How are extrammary Paget disease and Paget disease of the nipple different?
Answer
Extramammary Paget disease does not arise from any underlying carcinoma. By contrast, Paget disease of the nipple is is simply a manifestiation of underlying (usually ductal) carcinoma.
Card 319
How can extramammary Paget disease be distinguished from melanoma of the vulva?
Answer
Extramammary Paget disease is PAS⁺, keratin⁺, and S100⁻
Melanoma is PAS⁻, keratin⁻, S100⁺
Card 320
What embryological structures is the vaginal canal derived from, and what types of epithelium correspond to each? How do these epithelial types change during development
Answer
Lower 1/3 derived from urogenital sinus, associated with squamous epithelium.
Upper 2/3 derived from Müllerian ducts, associated with columnar epithelium.
During development, squamous epithelium from urogenital sinus grows upward to replace all columnar epithelium.
Card 321
What is adenosis and what exposure is it associated with? What malignancy does it predispose towards?
Answer
Adenosis = aberrant persistence of columnar epithelium in upper vagina.
Associated with in-utero exposure to diethylstilbestrol (DES) - a synthetic estrogen
Associated with clear cell carcinoma
Card 322
What is clear cell carcinoma?
Answer
Malignant proliferation of glandular tissue with clear cytoplasm, strongly associated with in utero exposure to DES
Card 323
Embryonal rhabdomyosarcoma is what type of tumor (what is the malignant cell)?
Answer
A malignant mesenchymal proliferation of immature skeletal muscle
Card 324
Embryonal rhabdomyosarcoma presents where, anatomically? What is another name for it?
Answer
Presents as a bleeding, grape-like mass protruding from the penis or vagina of a child <5 years.
"Sarcoma botryoides"
Card 325
What kind of cell is histologically characterized by cytoplasmic cross-striations and positive immunohistochemical stains for desmin and myogenin?
Answer
Rhabdomyoblast
Card 326
What are VIN and VAIN? What are their significance?
Answer
Vulvar intraepithelial neoplasia; vaginal intraepithelial neoplasia.
Both caused by high-risk HPV, and can lead to carcinoma of their respective organs.
Card 327
What lymph nodes does vaginal cancer typically spread to?
Answer
Cancer in the upper 2/3 spreads to the regional iliac nodes.
Cancer in the lower 1/3 spreads to the inguinal nodes.
Card 328
What are the 3 anatomical divisions of the cervix, and what are the relevant histological characteristics?
Answer
1) Endocervix - single layer of columnar cells
2) Transformation zone
3) Exocervix - nonkeratinizing squamous epithelium
Card 329
What is the result of most instances of HPV infection?
Answer
Total clearance by acute inflammation
Card 330
What is the consequence of a persistent HPV infection?
Answer
Cervical intraepitheial neoplasia (CIN)
Card 331
High risk HPV strains produce which two proteins which perfrom what respective functions?
Answer
E6 and E7, which respectively destroy p53 and Rb
Card 332
What are the stages of CIN severity?
Answer
CIN I: 1/3 thickness involved
CIN II: 2/3 thickness involved
CIN III: almost full thickness involved
Carcinoma in situ (CIS): full tickness involved
Card 333
Can CIN regress?
Answer
Yes - CIN I often regresses. However, the further the progression, the less likelihood of regression.
Card 334
How does cervical carcinoma typically present?
Answer
Vaginal bleeding (typically postcoital) or cervical discharge in a middle-aged woman (40-50)
Card 335
What are 2 important secondary risk factors (HPV being the primary) for cervical carcinoma?
Answer
Smoking; immunosuppression (cervical carcinoma is an AIDS-defining illness)
Card 336
What is a common cause of death in patients with cervical carcinoma, and how does it occur?
Answer
Hydronephrosis with postrenal failure; due to tumor invasion through the anterior uterine wall and into the bladder
Card 337
What percentage of cervical carcinoma cases are squamous cell carcinoma? What about adenocarcinoma?
Answer
80%;15%
(I'm aware this doesn't add up to 100%)
Card 338
How long does the progession from CIN to carcinoma typically take?
Answer
10-20 years
Card 339
When and how often are pap smears performed?
Answer
Age 21 on; every 3 years
Card 340
What is depicted in the pathology specimen shown below?
Answer
Pap smear specimen showing mid/high grade dysplasia (blue cells with hyperchromatic nuclei and increased N/C ratio)
Card 341
What is the protocol following an abnormal pap smear?
Answer
Confirmatory colposcopy with biopsy
Card 342
What are two key limitations of the pap smear?
Answer
1) Inadequate sampling of the transition zone leads to a high rate of false negative results
2) Has not affected the incidence of adenocarcinoma (doesn't go through CIN → CIS sequence)
Card 343
The quadrivalent HPV vaccine covers which strains of HPV?
Answer
6, 11, 16, 18
Card 344
What are the 3 stages of endometrial growth/shedding? How do hormones influence each?
Answer
1) Growth (proliferative phase) - estrogen driven
2) Preparation for implantation - progesterone driven
3) Shedding - loss of progesterone support
Card 345
What is Asherman syndrome?
Answer
Amenorrhea resulting from loss of the endometrial basalis (stem cell) layer. Usually a result of overaggressive dilation and curettage (D&C).
Card 346
What is an anovulatory cycle?
Answer
An estrogen-driven proliferative phase with no subsequent progesterone-driven secretory phase, due to lack of ovulation.
Common cause of dysfunctional uterine bleeding during menarche and menopause.
Card 347
What predisposes towards acute endometritis? How does it present?
Answer
Endometritis is typically caused by retained products of conception (post-delivery or abortion), which serve as a nidus for bacterial colonization.
Presents as a fever, uterine bleeding, and pelvic pain.
Card 348
Chronic endometritis is characterized by proliferation of what cell types into the endometrium?
Answer
Lymphocytes and plasma cells;
Plasma cells are necessary for the diagnosis, since lymphocytes are normally present in the endometrium anyway.
What undesirable outcome can chronic endometritis result in?
Answer
Infertility
Card 351
What is an endometrial polyp? How does it present? What drug commonly can cause it?
Answer
Hyperplastic protrusion of the endometrium;
Presents as abnormal uterine bleeding;
Can be caused by tamoxifen (estrogen agonist in the endometrium)
Card 352
What is endometriosis and what likely causes it?
Answer
Endometriosis refers to the presence of endometrial glands and stroma outside of the uterine endometrial lining.
Likely due to retrograde menstruation with implantation at an ectopic site.
Card 353
How does endometriosis present?
Answer
With dysmenorrhea and pelvic pain
Card 354
What are the most common sites of endometriosis and how does each site present (5)?
Answer
1) *Ovary - chocolate cyst (most common location)
2) Uterine ligaments - pelvic pain
3) Pouch of douglas - pain with defacation
4) Bowel serosa - adhesions
5) Fallopian tube mucosa - increased risk of ectopic pregnancy
Card 355
How do endometriosis implants typically appear, grossly?
Answer
Yellow-brown "gunpowder" nodules
Card 356
Endometrial involvement of the uterine myometrium is called what?
Answer
Adenomyosis
Card 357
There is an increased risk of ______ at the site of endometriosis, especially in the _______.
Answer
Carcinoma; ovary
Card 358
What is directly responsible for endometrial hyperplasia, and what are some causes of that direct cause?
What is the most important predictor for whether endometrial hyperplasia will progress to carcinoma?
Answer
Presence of cellular atypia:
Simple hyperplaia with atypia = 30% chance of progression
Complex hyperplasia without atypia = < 5% chance
Card 361
What is the most common invasive carcinoma of the female genital tract?
Answer
Endometrial carcinoma, a malignant proliferation of endometrial glands
Card 362
What are the two pathways of endometrial carcinoma development? What percentage of cases is attributable to each?
Answer
Hyperplasia (75%) and sporadic (25%)
Card 363
What are the risk factors for endometrial carcinoma arising from hyperplasia (4)?
Answer
1) Early menarche/late menopause
2) Nulliparity
3) Infertility with anovulatory cycles
4) Obesity
*All of these factors are related to increased exposure to estrogen
Card 364
Compare and contrast the hyperplasia and sporadic pathways of endometrial carcinoma development in terms of -
Age of onset:
Histology:
Genetics:
Answer
60 years; 70 years
Endometrioid histology; serous histology with papillary structures and psammoma bodies
MSI and PTEN mutations possible; p53 mutations common
Card 365
Which is more aggressive, endometrial carcinoma arising sporadically or from endometrial hyperplasia?
Answer
Sporadic pathway yields more agressive cancers.
Card 366
What is a leiomyoma? How common is it?
Answer
A benign neoplastic proliferation of smooth muscle arising from the myometrium. It is the most common tumor in females.
Card 367
What are three properties regarding the presentation and natural history of leiomyomas?
Answer
1) Common in premenopausal women
2) Multiple tumors are typically present
3) Enlarge during pregnancy and shrink after menopause
Card 368
Do leiomyosarcomas arise from leiomyomas?
Answer
NO. They arise de novo.
Card 369
In whom are leiomyosarcomas typically seen?
Answer
Postmenopausal women
Card 370
What 3 histological features will one see in a leiomyosarcoma?
Answer
1) Necrosis
2) Mitotic activity
3) Cellular atypia
*Basically the nondescript features of any solid tumor
Card 371
What is the functional unit of the ovary?
Answer
The follicle
Card 372
What role do LH, FSH, and estradiol play in ovulation?
Answer
1) LH acts on the theca cells of the follicle to induce androgen production
2) FSH stimulates granulosa cells to convert androgen to estradiol
3) Estradiol surge induces an LH surge (positive feedback loop) which triggers ovulation
Card 373
What is the architecture of a follicle?
Answer
Central oocyte surrounded by granulosa and theca cells
Card 374
What is the corpus luteum?
Answer
The post-ovulation residual follicle that secretes progesterone in order to prepare the endometrium for implantation.
Card 375
What pathology involving the corpus luteum is possible during early pregnancy?
Answer
Hemorrhage into the corpus luteum, resulting in a hemorrhagic corpus luteal cyst
Card 376
What is the clinical significance of follicular cysts in the ovary?
Answer
Small numbers are quite common in women and have no real clinical significance unless they become too numerous
Card 377
Describe the pathophysiology behind the development of polycystic ovarian disease (PCOD):
Answer
1) LH induces excess androgen production (hirsutism results)
2) Androgen is converted back to estrone in adipose tissue
3) Estrone feedback causes a decrease in FSH - cystic degeneration of follicles results
4) Circulating estrone levels increase risk for endometrial carcinoma
Card 378
What LH:FSH ratio characterizes PCOD?
Answer
LH:FSH ratio > 2
Card 379
What is the classic presentation of PCOD (3)?
Answer
Obese young woman with:
1) Infertility
2) Oligomenorrhea
3) Hirsutism
Card 380
What is a long-term complication of PCOD?
Answer
Development of insulin resistance and DM type 2 after 10-15 years
Card 381
What are the 3 cell types contained within the ovary?
What is the most common type of ovarian tumor (70% of cases)?
Answer
Surface epithelial tumor (derived from the coelomic epithelium lining the ovary)
Card 383
Coelemic epithelium produces the epithelial lining of which 3 female reproductive structures?
Answer
1) Fallopian tube
2) Endometrium
3) Endocervix
Card 384
What are the two types of surface epithelial tumor of the ovary, and what substances do they produce?
Answer
Serous tumor - filled with watery fluid
Mucinous tumor - filled with mucus
Card 385
Are mucinous tumors benign or malignant? Serous tumors?
Answer
Both types can actually be classified as "benign," "borderline," or "malignant."
"Serious" (e.g. BRCA1) tumors are more often serous
Card 386
Describe the gross appearance of a benign cystadenoma. In whom is this tumor most commonly found?
Answer
Single ovarian cyst with a flat, simple lining.
Common in premenopausal women (30-40 years old).
Card 387
Describe the gross appearance of a malignant cystadenoma. In whom are these tumors more common?
Answer
Complex cyst with a thick, shaggy lining. Common in women age 60-70.
*Papillary serous cystadenocarcinoma is the most common malignant cystadenoma and most common malignant ovarian tumor
Psammoma mnemonic:
papillary carcinoma of the thyroid,
serous cystadenocarcinoma of the ovary,
meningioma,
ₘₑₛₒₜₕₑₗᵢₒₘₐ
Card 388
What is the prognosis for a borderline cystadenoma?
Answer
Better than for a malignant cystadeoma, but still has the potential to metastasize.
Card 389
What are 2 less common types of surface epithelial ovarian tumor? What tissues do they possess and what is their malignant potential?
Cystic teratoma, the most common germ cell tumor (10% of cases), typically contains what?
Answer
Fetal tissue dervied from 2 or 3 embryologic layers (bone, skin, cartilage, gut, thyroid)
Card 397
Though cystic teratoma are typically benign, the presence of what indicates malignant potential (2)?
Answer
1) Immature tissue (e.g. neural)
2) Somatic malignancy (tumor cells that become cancerous, e.g. melanocytes that became melanoma)
Card 398
What is a struma ovarii?
Answer
A teratoma that is comprised almost entirely of thyroid tissue
Card 399
Dysgerminoma, the most common malignant germ cell tumor, has what kind of histology?
Answer
Large cells with clear cytoplasm and central nuclei (resembles oocytes)
Card 400
What is the testicular counterpoint of a dysgerminoma?
Answer
Seminoma
Card 401
What is the prognosis of a dysgerminoma? What is the treatment?
Answer
Good prognosis - responds to radiotherapy
Card 402
What marker might be elevated in cases of dysgerminoma?
Answer
Serum LDH (more commonly seen as a biomarker for dysgerminoma in children)
Card 403
Endodermal sinus tumors - the most common germ cell tumor in children - presents with what elevated biomarker? What is the classic finding on histology?
Choriocarcinoma is comprised of what kinds of cells? How is it distinguishable from placental tissue?
Answer
Cytotrophoblasts, syncytiotrophoblasts.
Mimics placental tissue except for loss of villi.
Card 405
What biomarker is commonly elevated in choriocarcinoma, and what pathology does this biomarker actually induce?
Answer
β-hCG; leads to thecal cysts in the ovary
Card 406
What is the clinical significance of choriocarcinoma that arises as an ovarian germ cell tumor as opposed to other source tissues?
Answer
Much poorer response to chemotherapy
Card 407
2 features of embryonal carcinoma?
Answer
1) Composed of large, primitive cells
2) Highly agressive, early metastasis
Card 408
What are the 3 sex cord-stromal tumors (in females)?
Answer
1) Granulosa-theca tumors
2) Sertoli-Leydig cell tumor
3) Fibroma
Normal sex cord histology:
Card 409
What 3 signs can a granulosa-theca tumor present with? What underpins all of these symptoms?
Answer
1) Precocious puberty (if tumor occurs prior to puberty)
2) Menorrhagia or metrorrhagia (if tumor occurs at reproductive age)
3) Endometrial hyperplasia and inappropriate uterine bleeding (if tumor occurs postmenopausally)
*All of these presenting symptoms occur due to inappropriate estrogen excess
**Note: This passage from Pathoma is not phenomenally accurate. Dr. Kalir would distinguish between granulosa cell tumors and theca tumors, and mention specifically that granulosa tumors are likely to secrete androgens that lead to hirsutism and virilization, while theca tumors are more likely to secrete estrogen.
Card 410
Are granulosa-theca cell tumors malignant?
Answer
Technically yes, but they rarely metastasize
Card 411
Reinke crystals are a characteristic histologic feature of what type of tumor? What are the symptoms of this tumor?
Answer
Sertoli-Leydig tumor - Reinke crystals are found within Leydig cells.
Hirsutism and virilization (due to excess androgen production)
Card 412
Fibroma - a benign tumor of fibroblasts - is associated with what syndrome?
Answer
Fibroma can cause Meigs syndrome, which is characterized by ascites and pleural effusions
Card 413
What are two significant ovarian/peritoneal metastases?
Answer
1) Krukenberg tumor (diffuse type gastric adenocarcinoma)
2) Pseudomyxoma peritonei (AKA "Jelly Belly") - arises from mucinous tumor of the appendix (can also more infrequently come from mucinous tumor of the ovary).
Card 414
What is the major risk factor for ectopic pregnancy and what is the major presenting symptom?
Answer
Fallopian tube scarring (typically secondary to endometriosis or PID)
Lower quadrant abdominal pain a few weeks after a missed period - very key to include this in the differential for a female presenting with signs of appendicitis.
Card 415
When and how often do spontaneous abortions occur?
Answer
Before 20 weeks gestation;
25% of all pregnancies
Card 416
What are 4 causes of spontaneous abortion?
Answer
1) Chromosomal abnormalities (trisomy 16 - memory tip: sleeping beauty fated to die at age 16)
2) Hypercoagulable states (antiphospholipid syndrome)
3) Congenital infection (ToRCHES)
4) Teratogens
Card 417
Effects of teratogens generally depends on the dose, agent, and time period of exposure. What effects are seen in exposure during the: first two weeks, weeks 3-8, and months 3-9?
Answer
0 - 2 weeks: abortion
3 - 8 weeks: risk of organ malformation
3 - 9 months: risk of organ hypoplasia
Card 418
What is placenta previa? How does it present and how is it treated?
Answer
1) Implantation of the placenta in a position that overlies the cervix
2) Presents as 3rd trimester bleeding
3) Requires delivery by caesarian section
Card 419
What is a placental abruption? How does it present? What is a common consequence?
Answer
1) Separation of the placenta from the decidua prior to delivery of the fetus.
2) Present with third trimester bleeding
3) Commonly causes stillbirth
Card 420
What is placenta accreta? How does it present? How must it usually be treated?
Answer
Improper implantation of the placenta to the myometrium with little/no intervening decidua;
difficulty delivering the placenta & postpartum bleeding;
requires hysterectomy
Card 421
Teratogenic effects of alcohol?
Answer
Common cause of mental retardation;
leads to facial and cardiac abnormalities (e.g. VSD; neural crest cells involved in both face and heart development),
microcephaly
Card 422
Teratogenic effects of cocaine?
Answer
Intrauterine growth retardation and placental abruption
Card 423
Teratogenic effects of thalidomide?
Answer
Limb hypoplasia/defects
Card 424
Teratogenic effects of cigarette smoke?
Answer
Intrauterine growth retardation
Card 425
Teratogenic effects of isoretinoin?
Answer
Spontaneous abortion; hearing/vision impariment
Card 426
Teratogenic effects of tetracycline?
Answer
Discolored teeth
Card 427
Teratogenic effects of warfarin?
Answer
Fetal bleeding
Card 428
Teratogenic effects of phenytoin?
Answer
Digit hypoplasia; cleft lip/palate
Card 429
What 3 symptoms define preeclampsia, when does it arise, and in how many women?
Answer
1) Pregancy-induced hypertension, proteinuria, and edema
2) Arises in the 3rd trimester
3) 5% of pregancies
Card 430
What kind of symptoms will patients with preeclampsia complain of?
Answer
Headaches, visual disturbances
Card 431
What causes preeclampsia and how does it resolve?
Answer
Preeclampsia is due to an abnormality of the maternal-fetal vascular interface in the placenta;
it resolves with delivery
Card 432
What distinguishes eclampsia from preeclampsia?
Answer
Eclampsia is preeclampsia with seizures
Card 433
What is HELLP? What does the acronym stand for?
Answer
HELLP is preeclampsia with thrombotic microangiopathy involving the liver.
Hemolysis, Elevated Liver enzymes, Low Platelets
Card 434
What is the best way to treat both eclampsia and HELLP?
Answer
Immediate delivery
Card 435
During what age are infants susceptible to SIDS?
Answer
1 month - 1 year
Card 436
What are 3 risk factors for SIDS?
Answer
1) Sleeping on stomach
2) Exposure to cigarette smoke
3) Prematurity
Card 437
What is a hydatidiform mole? What kind of cells is it comprised of?
Answer
An abnormal conception chracterized by swollen and edematous villi with a proliferation of trophoblasts.
Card 438
How are partial and complete hydatidiform moles genetically different?
Answer
Complete: empty ovum fertilized by 2 sperm, 46 chromosomes (or 1 sperm + duplication)
Partial: normal ovum fertilzed by 2 sperm, 69 chromosomes
Card 439
Is fetal tissue present in a partial hydatidiform mole or a complete mole?
Answer
Fetal tissue is only present in a partial mole
Card 440
How are the patterns of villous edema differently characterized between a partial and complete hydatidiform mole?
Answer
Partial mole: some villi are hydropic, some are normal
Complete mole: most villi are hydropic
Card 441
How are the patterns of trophoblastic proliferation different in partial and complete hydatidiform moles?
Answer
Partial mole: focal proliferation around hydropic villi only
Complete mole: diffuse, circumferential proliferation around hydropic villi
Card 442
What is the risk for progression to choriocarcinoma in a partial hydatidiform mole? A complete mole?
Answer
Partial: virtually no risk
Complete: 2-3% progress
Card 443
How does a hydatidiform mole present?
Answer
Uterus expands as if pregnancy is normal, but uterus soon becomes much larger and β-hCG becomes much higher than expected for the date of gestation
Card 444
What is the classic appearance of a hydatidiform mole on ultrasound?
Answer
"snowstorm appearance"
Card 445
If a hydatidiform mole goes undected through the second trimester, how can it present?
Answer
Passage of grape-like masses throught the vaginal canal.
Card 446
How do choriocarcinomas that arise from the gestational pathway (a hydatidiform mole) differ from those that arise from the germ cell pathway?
Answer
These choriocarcinomas (from the gestational pathway) respond very well to chemotherapy
Card 447
What is depicted here in this histological specimen?
Answer
This glomerulus-like structure is a "Schiller-Duval body", and is classic for an endodermal sinus tumor
Card 448
What kind of tumor did this specimen likely come from?
Answer
A dysgerminoma
Card 449
What is depicted here?
Answer
Hydropic villi characteristic of a hydatidiform mole
Card 450
This histological speciment represents what pathology?
Answer
Extramammary paget disease of the vulva (note the proliferation of malignant epithelial cells into the epidermis)
Card 451
What does this vaginal histology specimen depict?
Answer
Vaginal adenosis
Card 452
What pathology does this specimen depict?
Answer
Trick question: no pathology - this is a normal cervical transition zone
Card 453
What is the difference between hypospadias and epispadias (location, embryological etiology)? What is epispadius associated with?
Answer
Hypospadius: opening of urethra on inferior (ventral) surface of the penis; due to failure of the urethral folds to close.
Epispadius: opening of urethra on superior (dorsal) surface of penis; due to abnormal position of the genital tubercle. This is associated with bladder exstophy
Card 454
What serotypes of Chlamydia trachomatis cause lymphogranuloma venereum? What is lymphogranuloma venereum?
Answer
Serotypes L1-L3. Lymphogranuloma venereum is a necrotizing granulomatous inflammation of the of the inguinal lymphatics and nodes.
Card 455
What are 3 precursor lesions for squamous cell carcinoma of the penis?
Answer
Bowen disease: in situ carcinoma of the penile shaft or scrotum; presents as leukoplakia
Erythroplasia of Queyrat: in situ carcinoma of the glans that presents as erythroplakia
Bowenoid papulosis: in situ carcinoma that presents as multiple reddish papules and usually does not become invasive (unlike other two). Presents at a younger age (40s)
Card 456
What pathogens cause orchitis in young adults, older adults, and teens?
Answer
Young adults: Chlamydia trachomatois (serotype D-K) or Neisseria gonorrhea.
Older adults: E. coli and Pseudomonas
Teenage males: Mumps
Card 457
What vein can renal cell carcinoma invade leading to a "bag of worms" appearance of the scrotum? What is the "bag of worms"?
Answer
It invades the LEFT renal vein; varicocele
*Important to remember that the right testicular/ovarian vein drains directly into the IVC. Therefore, renal cell carcinoma invasion of the right renal vein will not produce varicocele.
Card 458
In a hydrocele, in which layer of the scrotum does fluid collect?
Answer
Tunica vaginalis
Card 459
Describe a seminoma? Does it respond to radiotherapy?
Answer
Large cells with clear cytoplasm and central nuclei (just like a dysgerminoma); homogeneous mass with NO hemorrhage or necrosis. Good prognosis; responds to radiotherapy.
Card 460
If there is ↑ AFP or β-hCG and a testicular mass, what might you suspect? What can chemotherapy do to this type of mass?
Answer
Embryonal carcinoma, a malignant tumor made of up immature, primitive cells that may produce glands.
Chemotherapy may result in differentiation into another type of germ cell tumor (e.g. teratoma)
*Note that teratomas can also secrete AFP or β-HCG. Apologies for the vagueness of the question
Card 461
What is the most common testicular tumor in children?
Answer
Yolk sac (endodermal sinus) tumor; AFP is characteristically elevated.
Weird/Desperate Memory Tip:
Associate sunny-side-up eggs (and yolk) with kids
Card 462
A male has a choriocarcinoma and presents with both gynecomastia and hyperthyroidism. Why is he presenting with these issues?
Answer
Choriocarcinoma secretes β-hCG, and the α-subunit of hCG is similar to that of FSH, LH and TSH.
Ergo, hCG activates TSH receptors → hyperthyroidism;
hCG also activates FSH/LH receptors → gynecomastia
Card 463
Are teratomas in males malignant? What can they secrete?
Answer
They're malignant in postpubertal males, but not in children. They can secrete AFP or β-hCG.
Card 464
If a man presents with a testicular mass that has Reinke crystals on histology what tumor is it? What can this tumor produce, and what secondary effects can it cause in children and adults?
What is the most likely cause of a 75 year old man's bilateral testicular mass?
Answer
Lymphoma; usually diffuse large B cell (DLBCL).
Card 466
What are the most common pathogens in acute prostatitis in young adults vs older adults? How does the prostate present?
Answer
Young adults: Chlamydia trachomatis and N. gonorrhoeae.
Older adults: E. coli and Pseudomonas.
Prostate: tender and boggy
Card 467
What is the hormone that drives BPH?
Answer
Dihydrotestosterone (DHT)
Card 468
Where is the most common location of prostate adenocarcinoma?
Answer
Peripheral posterior zone of the prostate
Card 469
What is the characteristic look of the cells in prostate adenocarcinoma?
Answer
Infiltrating glands; nuclei that have dark nucleoli.
Card 470
What drugs can be used to reduce testosterone conversion to DHT?
Answer
5α-reductase inhibitors:
finasteride,
dutasteride
Memory Tips:
"finesse" and "detest" are less masculine, i.e. finasteride/dutasteride block production of the more potent androgen DHT
Card 471
What is depicted in the histology slide below?
Answer
Koilocytic changes due to HPV 6 or 11
Card 472
What is testicular torsion? What is the usual predisposing factor? Who does it present in and how?
Answer
Twisting of the spermatic cord leading to hemorrhagic infarction.
Can be due to congenital failure of testes to attach to inner lining of the scrotum (via the processus vaginalis)
Presents in adolescents with sudden testicular pain and absent cremaster reflex
Card 473
What is a varicocele? How does it present? What malignancy is associated?
Answer
Dilation of the spermatic vein due to impaired drainage of the pampiniform plexus.
Presents as scrotal swelling with "bag of worms" appearance - often associated with infertility.
Usually left sided -- associated with renal cell carcinoma obstructing left renal vein
Card 474
What is a hydrocele?
What are the two distinct pathophysiologic causes for children and adults?
Can it be transilluminated?
Answer
Fluid collection within the tunica vaginalis.
Associated with incomplete closure of processus vaginalis (infants) or blockage of lymphatic drainage (adults).
Presents as scrotal swelling that can be transillluminated.
Card 475
What does this histology specimen depict?
Answer
Seminoma (or dysgerminoma - they look the same)
Card 476
What kind of pathology is this gross speciment typical of?
Answer
Seminoma. Homogeneous mass. No hemorrage. No necrosis.
Card 477
What malignancy is depicted?
Answer
Embryonal carinoma of the testes. Note the glandular formation.
Card 478
What is the most common testicular tumor in children? What biomarker is elevated? What histological feature may be present?
Choriocarcinoma mimics placental tissue. What differentiates it from the placenta?
Answer
Placental tissue possesses vascularized villi. Cytotrophoblasts and syncytiotrophoblasts sit around the villi. In choriocarcinoma, there are no blood vessels. It is only made up of the trophoblasts themselves.
Card 480
What malignancy is depicted below? What two cell types are identifiable?
Answer
Choriocarcinoma.
Small circles: Cytotrophoblasts.
Bigger circles: Syncytiotrophoblasts (multinucleated)
Card 481
Identify the Sertoli and the Leydig cells in this normal histological specimen of the sex-chord stroma:
What can be seen in this histopathological specimen?
Answer
Reinke crystals, characteristic of a Leydig Cell Tumor
Card 483
What is apparent in this histology specimen taken from the prostate?
Answer
Small glands of adenocarcinoma in the prostate. Nuclei (circled structures) contain dark nulceoli.
Card 484
What drugs can be used in the treatment of prostate adenocarcinoma?
Answer
Continuous GnRH analogs (e.g., leuprolide) shut down the hypothalamus ⇒ ↓ LH and FSH
Flutamide acts as a competitive inhibitor at the androgen receptor.
Memory Tips:
Leuprolide breaks the cyclic "loop" of GnRH secretion;
-tamide drugs block testosterone to make cancer more "timid"
Card 485
What are condyloma acuminatum?
Answer
Benign warty growths due to HPV 6 and 11
Card 486
Perianal involvement of lymphogranuloma venereum can lead to what complication?
Answer
Fibrosing rectal stricture
Card 487
What are two risk factors for the development of squamous cell carcinoma of the penis?
Answer
1) High risk HPV (2/3 of cases)
2) Lack of circumcision - foreskin acts a nidus
Card 488
What is cryptorchidism?
Answer
Failure of the testicle to descend into the scrotal sac.
Card 489
What are three complications of cryptorchidism?
Answer
1) Testicular atrophy
2) Infertility
3) Increased risk for seminoma
Card 490
Granulomas involving the seminiferous tubules are characteristic of what type of orchitis?
Answer
Autoimmune orchitis
Card 491
How can testicular tumors be differentiated from hydrocele?
Answer
They cannot be transilluminated
Card 492
From what two general lineages can testicular tumors arise from? Which is more common?
Answer
Germ cells or sex cord stroma. Most testicular tumors arise from germ cells.
Card 493
Why would you biopsy a testicular tumor?
Answer
You would not - they are almost never biopsied for fear of seeding the scrotum
Note: excise via radical orchiectomy, but don't biopsy due to risk of seeding scrotum; majority are germ cell tumors anyway
Card 494
How are testicular tumors typically removed?
Answer
Radical orchiectomy
Note: excise via radical orchiectomy, but don't biopsy due to risk of seeding scrotum; majority are germ cell tumors anyway
Card 495
What pathology is evident in this gross speciment of the testicle?
Answer
Embryonal carinoma of the testes
Card 496
What is the common age of presentation of a testicular germ cell tumor?
Answer
15-40 years of age
Card 497
What are two risk factors for developing a testicular germ cell tumor?
Answer
1) Cryptorchidism
2) Klinefelter syndrome
Card 498
How are testicular germ cell tumors first characterized? How is the natural history of disease caused by each different?
Answer
Seminoma (55% of cases) - highly responsive to radiotherapy and metastasize late
Non-seminoma (45%) - variable response to treatment, metastasize early
Card 499
A seminoma is the counterpart of which female genital tract malignancy?
Answer
Dysgerminoma
Card 500
How can seminoma and embryonal carcinoma be grossly distinguished?
Answer
Embryonal carcinoma forms a hemorrhagic mass with necrosis, whereas
seminoma does not hemorrhage or necrose