3.06, 4.07, 4.09, 4.18 Exam 2 Pathology

Question 1

> 2cm mass in colonic mucosa that is sessile (no stalk) and villous histology

Answer 1

neoplastic adenoma polyp (malignant potential)

Question 2

protrusion of colonic mucosa with serrated appearance upper 1/3 crypt on microscopy, <5mm, left sigmoid colon + rectum, more frequent seen in multiples

Answer 2

HP: non-neoplastic hyperplastic polyps

Question 3

Sessile large, right sided colon polyps with dilation, branching, and serration extending to the base with characteristic T and L shapes

Answer 3

SSA/P: sessile serrated adenoma/polyp

Question 4

chronic intermittent rectal bleeding, mucus discharge, inflammatory polyps on anterior rectal wall that shows well demarcated ulcer with congestion, hemorrhage, and mucoid exudates on colonoscopy

Answer 4

SRUS single rectal ulcer syndrome

Question 5

90% of all polyps in children <5 yo, solitary, hamartomatous, no increased cancer risk

Answer 5

sporadic juvenile polyp

Question 6

child with rectal bleeding, anemia, diarrhea, intussusception, intestinal obstruction, or polyp prolapse that on coloscopy shows but there are multiple polyps or multilobulated (at least 5) in stomach and colon or (+) FH, almost always syndromic with cardiac, vascular, skeletal, cranial deformities and malrotations/diverticulum’s

Answer 6

JPS: juvenile polyposis syndrome
increased risk of colorectal carcinoma

Question 7

20% of JPS patients have germline mutations in what gene and shows up with what stain?

Answer 7

SMAD4 gene + immunostain

Question 8

11 yo with mucocutaneous hyperpigmentation, hematochezia, hematemesis, abdominal pain, hamartomatous polyps, arborization and presence of smooth muscle mixed into lamina propria, and increased cancer risk

Answer 8

Peutz-Jeghers Syndrome

Question 9

What inheritance pattern and mutation in 70-80% Peutz-Jegher’s Syndrome?

Answer 9

AD, LKB1/STK11

Question 10

what is the inheritance pattern and mutation for familial adenomatous polyposis?

Answer 10

AD, APC gene

Question 11

25-year-old colonoscopy shows over 100 polyps, family members that died of colorectal cancer, and carry APC mutation, next step?

Answer 11

FAP - prophylactic colectomy

Question 12

most common neoplastic polyp and why all adults in the US recommended colonoscopy after 45 yo?

Answer 12

colonic adenoma

Question 13

Does a single cancerous colonic polyp increase the risk of cancer in another polyp?

Answer 13

nope

Question 14

What colonic adenoma architecture is most likely to harbor cancers?

Answer 14

villous adenomas (larger sessile) > tubular

Question 15

An older adult with what is assumed to have colorectal cancer until proven otherwise?

Answer 15

iron deficiency anemia

Question 16

65 yo comes in with asymptomatic chronic bleeding, iron deficiency anemia, positive fecal occult blood test, colonoscopy shows a “raised lesion” in the ascending colon, while biopsy reveals infiltrative malignant glands, and desmoplasia (submucosal invasion)?

Answer 16

colon adenocarcinoma “right sided”

Question 17

What is the most common variant of colorectal carcinoma?

Answer 17

intestinal type adenocarcinoma 90%

Question 18

If 75 yo come after years of asymptomatic chronic bleeding, iron deficiency anemia, positive fecal occult blood test now experience endocarditis (streptococcus bovis positive), ascites, SBO, bladder symptoms, abdominal guarding, and sciatic nerve pain? What may be elevated on serum testing?

Answer 18

metastasis of colorectal adenocarcinoma (+ enterocutanous fistulas)
bad prognosis = risk of death
CEA serum elevated

Question 19

Biopsy of intestines reveals MICRO transmural inflammation, lymphoid aggregates with granulomas, neutrophils in the epithelium, basal lymphoplasmacytosis
GROSS skip lesions, predisposition for strictures, fistulas, and cobblestone mucosa?

Answer 19

crohn’s

Question 20

What form of IBD is most associated with an increased risk of neoplasia?

Answer 20

Ulcerative colitis

Question 21

Biopsy of intestines reveals MICRO normal serosal surface, lymphoplasmacytic inflammation of the lamina propria, basal plasmacytosis, crypt abscesses, inflammation restricted to the mucosa, and intact muscularis
GROSS no strictures or mural thickening BUT pseudopolyps, hemorrhagic ulcerations, mucosal bridges

Answer 21

ulcerative colitis

Question 22

chronic inflammation leading to damaged muscularis propria and disturbed neuromuscular function leading to colonic dilation and risk for perforation

Answer 22

toxic megacolon

Question 23

Patients present with diarrhea, flushing, and asthmatic symptoms; they also present with RLQ pain. What do you need for a risk assessment?

Biopsy of mass would reveal a yellow nodule around the area of the mural thickening with “salt and pepper: nuclear chromatin, serotonin positive, and chromogranin positive, synaptophysin stain

Answer 23

carcinoid syndrome from a well-differentiated neuroendocrine tumor on the appendix
Ki-67 labeling index

Question 24

What are most NET tumors of the GI made up of, what do they secrete, and how can you diagnose them?

Answer 24

enterochromaffin cells, G cell tumors, and rectal trabecular L cells
secrete serotonin
diagnose with >10mg 5-hydroxyindolacetic acid in 24 hour urine collection

Question 25

Usually asymptomatic and found incidentally, it can lead to appendiceal rupture as it fills with mucus, and ovarian involvement is common

Answer 25

Appendiceal Mucinous Neoplasm (AMN)

Question 26

what is the genetic defect associated with congenital hyperbilirubinemia?

Answer 26

UGT1A1 gene

Question 27

What is the most common congenital hyperbilirubinemia?

Answer 27

Gilberts Syndrome

Question 28

13 year old male with mild isolated unconjugated hyperbilirubinemia and normal LFTs, caloric restriction, and certain drugs (acetaminophen and rifampin) make it worse. He was previously diagnosed with chronic hepatitis but it doesn't seem right

Answer 28

Gilbert's Syndrome AR, UGT1A1*Var. 28

Question 29

10 yo female with severe unconjugated hyperbilirubinemia (>25mg) at birth. Phototherapy reduced levels but phenobarbital did nothing. There were no detectable UDP-GT or activity expression in hepatic tissue. At 1 year old had an encephalopathic attack (kernicterus). She was placed on the liver transplant list.

Answer 29

Crigler-Najjar Syndrome (Type 1) AR, UGT1A1 complete loss of function

Question 30

15 yo female with moderate serum unconjugated hyperbilirubinemia (5-20mg) following severe UTI and treatment. Phototherapy and phenobarbital were effective as treatment?

Answer 30

Crigler-Najjar Syndrome (Type 2) AD, UGT1A1 partial deficiency

Question 31

A pregnant patient with intermittent jaundice, serum elevated conjugated hyperbilirubinemia due to reduced glutathione excretion, and mild RUQ pain. Undergoes liver exploratory biopsy that shows a "blackened liver", iron stain negative, with coarse granular brown-black pigment in hepatocytes and electron-dense lysosomal granules due to auto-oxidation (epi, tyr, p-ala, tryp). What is the defect and condition? Treatment?

Answer 31

Dubin Johnson Syndrome AR, CMOAT/MRP2/ABCC2 defect No TX, good prognosis

Question 32

Patient with photosensitivities and liver dysfunction, liver biopsy reveals fine golden brown pigmentation with protoporphyrin crystals in hepatocytes and bile canaliculi. What is the defect and condition?

Answer 32

Erythropoietic Protoporphyria ferrochelatase enzyme defect

Question 33

A patient with intermittent jaundice, serum elevated conjugated hyperbilirubinemia and mild RUQ pain. Undergoes liver exploratory biopsy that shows normal pigmentation of the liver. What is the defect and condition? Treatment?

Answer 33

Rotor Syndrome AR, organic anion storage defect No Tx/Benign

Question 34

Obstruction at large hepatic veins or the IVC?

Answer 34

budd chiari syndrome (BCS)

Question 35

obstruction at sinusoids or small hepatic veins

Answer 35

sinusoidal obstruction syndrome (SOS)

Question 36

Patient who recently underwent chemotherapy 3 months ago comes in with painful hepatomegaly, mild jaundice, and ascites. Their alkaline phosphatase was elevated. Biopsy of liver revealed sinusoidal dilation, congestion, hepatic plate atrophy, and RBC extravasation at space of Disse. There were signs of hepatocellular necrosis with absent inflammation.

Answer 36

subacute HVOO or Sinusoidal Obstruction Syndrome (SOS)

Question 37

A patient struggling with chronic liver failure for years undergoes a routine liver biopsy. Their transaminases are mildly elevated and alkaline phosphatase is elevated. It reveals fibrosis around teh central vein (pericentral) and sinusoids that progressed to cirrhosis that extended to teh hepatic and portal veins.

Answer 37

chronic HVOO, secondary sinusoidal thrombosis

Question 38

Rare hepatic venous outflow obstruction characterized by acute liver failure?

Answer 38

fulminant HVOO

Question 39

Patient with caput medusae, splenomegaly, hemorrhagic hepatic necrosis, and PMH of polycythemia vera?

Answer 39

Budd-Chiari Syndrome

Question 40

A patient with an allogenic stem cell transplant 3 weeks ago comes in experiencing hepatic congestion, portal hypertension, and early signs of liver failure. Liver biopsy reveals accumulation of debris in terminal hepatic vein, damage to endothelial cells in sinusoids, RBX extravasation, and necrosis of perivenular hepatocytes

Answer 40

Sinusoidal Obstruction Syndrome

Question 41

Who is at risk of SOS?

Answer 41

recent stem cell transplant chemotherapy "Bush tea" ingestion (crotalaria, senecio)

Question 42

What can lead to passive congestion of the liver?

Answer 42

right-sided heart failure

Question 43

What fails (cardiopulmonary) when you see sudden or short-term impairment of venous drainage and mottled appearance of congested areas of the liver?

Answer 43

right-sided heart failure/PE

Question 44

What fails (cardiopulmonary) when you see fibrosis, changes in liver architecture, and nutmeg appearance due to chronic pooling of sinusoid blood over a long time?

Answer 44

right-sided heart failure/constrictive pericarditis

Question 45

Patient going into shock with mottled nutmeg liver, pale periportal parenchyma, centrilobular hemorrhagic necrosis: what (cardiopulmonary) condition?

Answer 45

left-sided heart failure/shock

Question 46

If on a liver biopsy, you see brownish bile pigment with dilated canaliculi, blunted or fewer microvilli, bile accumulations in hepatocyte lysosomes, apoptotic cells, and kupffer cells with bile pigments what should you be suspecting?

Answer 46

cholestasis

Question 47

46 yo female comes in with mild jaundice, pruritus, skin xanthomas, and diarrhea with trouble seeing at night?

Answer 47

cholestasis (Vit A def.)

Question 48

liver biopsy reveals hepatic scarring and regenerative nodule formation with feathery triad of degeneration of hepatocytes including swelling, diffuse bile pigmentation, and reticulated appearance

Answer 48

chronic biliary obstruction

Question 49

What can be superimposed on the chronic biliary obstruction process triggering an acute-on-chronic liver failure?

Answer 49

ascending cholangitis

Question 50

Patient comes in septic experiencing RUQ pain, fever, and jaundice (Charcot triad). A bacterial culture reveals gram-negative bacteria. Where was the likely source of the infection and what will you find Gross + Micro?

Answer 50

Ascending cholangitis Gross: pus in bile ducts Micro: periductular neutrophils infiltration bile duct

Question 51

What clinical symptoms indicate suppurative cholangitis?

Answer 51

hypotension + mental confusion severe prognosis

Question 52

fibrous bands deposited by activated stellate cells around regenerative nodules of hepatocytes due to persistent necrosis with a characteristic "jigsaw" pattern that alters the liver architecture secondary to chronic bile duct obstruction (end-stage liver will be bile-stain color)?

Answer 52

secondary or obstructive biliary cirrhosis

Question 53

55 yo female comes in with insidious onset pruritis, progressive jaundice, and fatigue. She had been previously diagnosed with Sjogren's, abnormal thyroid, AMA (+), elevated IgM, eand levated bilirubin, radiology came back normal, and examination of bile ducts revealed florid duct lesions (with granulomas), cholestasis, and "jigsaw" nodule cirrhosis close to portal tracts due to due to nonsuppurative inflammation.

Answer 53

primary biliary cirrhosis (PBS) - Autoimmune

Question 54

23 yo male with unpredictable but progressive jaundice, pruritis, hepatomegaly, splenomegaly, diarrhea, and weight loss. He also struggles with IBD (ulcerative colitis) and episodes of pancreatitis. Elevated serum ANCA (+), GGT activity, and ATLs. Cholangiogarohy reveals the beading of large bile ducts of the biliary tree. Micro shows concentric "onion skin" fibrosis around bile ducts (periductal) with biliary "jigsaw: cirrhosis (aka a fibro-obliterative lesion). The liver upon gross examination is irregular, and firm, with diffuse variable-sized green nodules. What does he have and what is he at risk for?

Answer 54

primary sclerosing cholangitis (PSC) cholangiocarcinoma risk

Question 55

Chinese man who recently immigrated details recurring bouts of gallbladder duct infections (ascending cholangitis) leading to progressive inflammatory destruction of the liver parenchyma?

Answer 55

primary hepatolithiasis (intrahepatic gallstone formation)

Question 56

What will around for the content and color of most gallstones in the developed world?

Answer 56

cholesterol - yellow

Question 57

Choledocholithiasis describes what?

Answer 57

stones in the common bile duct

Question 58

cholelithiasis describes what?

Answer 58

stones in gallbladder

Question 59

What are some cholesterol gallstone risk factors?

Answer 59

age, obesity, familial, estrogen/OPC, hypertriglyceridemia, Hispanic women and native Americans

Question 60

What makes up large (>5mm), soft, flaky, BROWN bilirubin gallstones?

Answer 60

calcium salt bilirubin + palmitate + high [cholesterol]

Question 61

What kind of gallstones are most associated with ascending cholangitis, biliary inflammation, E. coli + fluke infections?

Answer 61

brown bilirubin gallstones

Question 62

What makes up the shiny, irregular, 2-5mm, BLACK gallstones?

Answer 62

calcium bilirubinate

Question 63

What kind of gallstones are most associated with older and malnourished individuals with chronic hemolysis, biliary infections, and total parental nutrition?

Answer 63

black bilirubin gallstones

Question 64

Patient comes in with RUQ pain and flatulence that comes on after eating steak or loaded nachos, what will confirm the diagnosis?

Answer 64

Radiograph for radiolucent gall stones (cholesterol) US if >3mm

Question 65

What are possible complications of cholelithiasis?

Answer 65

acute/chronic cholecystitis choledocholithiasis fistulas acute pancreatitis gallbladder cancer

Question 66

Patient who recently underwent immunosuppression post kidney transplant has RUQ pain, tenderness, fever, and guarding. Serum WBC, leucocytosis, and elevated alkaline phosphatase. PE shows (+) Murphey sign. Micro examination shows sludge formation and mucosal ischemia due to phospholipase release with increased lysolecithin and cholesterol-saturated bile.

Answer 66

Acute Cholecystitis

Question 67

Patient comes in with episodic, steady, abdominal pain located in the epigastrium or sometimes the RUQ that is brought on after eating meals. US shows wall thickening and fibrosis. Biopsy of affected tissues shows predominantly lymphocytic infiltrate and mucosal herniation into the muscularis propria (Rokitansky-Aschoff sinuses)

Answer 67

Chronic cholecystitis

Question 68

Patient comes in with symptoms of an SBO. Radiograph shows pneumobilia and calcification in the GI tract with proximal dilation and distal narrowing. (Rigler triad)

Answer 68

Gallstone Ileus (fistula to distal ileum)

Question 69

When a gallstone is the duodenum or the stomach?

Answer 69

Bouveret syndrome

Question 70

What is a common chronic cholelithiasis complication aka "porcelain gallbladder"?

Answer 70

GB cancer or cholanguiocarcinoma

Question 71

Patient comes in with abdominal pain, jaundice, anorexia, nausea, and vomiting. They have a history of chronic parasitic infections (East Asian) OR gallstones with porcelain gallbladder. imaging reveals a focal mural mass invading the adjacent liver and obstructing teh common bile duct. Biopsy shows glandular-like tissue with infiltrating fibrotic wall tissue and exophytic growth patterns.

Answer 71

Gallbladder adenocarcinoma

Question 72

what triggers the premature activation of enzymes that leads to the aberrant release and autodigestion of the pancreatic parenchyma leading to liquefactive necrosis and fat necrosis of pancreas?

Answer 72

trypsin --> acute pancreatitis

Question 73

what is the most common cause of acute pancreatitis?

Answer 73

gallstones

Question 74

A common cause of pancreatitis with toxic etiology?

Answer 74

alcohol

Question 75

saponification of fat tissue with fat necrosis that may extend to the omentum, retroperitoneum, bone marrow, and subcutaneous tissue; hemorrhaging, and necrotic areas that involve the duct lamina?

Answer 75

severe acute pancreatitis

Question 76

Enlarged and swollen pancreas with foci of fat necrosis (yellow-white/chalky) that recovers in 5-7 days

Answer 76

mild acute pancreatitis

Question 77

patient comes in with periumbilical and flank hemorrhage signs, epigastric pain that radiates to the back, necrosis that spreads from periumbilical soft tissue and retroperitoneum, elevated serum lipase and amylase, and hypocalcemia

Answer 77

acute pancreatitis

Question 78

A 45-year-old man with a history of alcohol abuse comes in experiencing abdominal pain, weight loss, nausea, vomiting, jaundice, ascites, DM, and steatorrhea. Reduced levels of serum vitamin K.

Answer 78

chronic pancreatitis

Question 79

28 yo male came in with abdominal pain, weight loss, nausea, vomiting, ascites, DM, and jaundice. They note recurrent attacks of similar pain in their youth. What genetic defect would you look for to confirm the diagnosis?

Answer 79

PRSS1 trypsinogen gene - chronic pancreatitis

Question 80

What is a serum marker with high Sp for pancreatic cancer?

Answer 80

CA19-9 >1000 U/mL

Question 81

Irregular atrophy and obliteration of pancreatic acini and ducts + fibrosis BUT normal lobular pancreatic architecture + preserved islets of Langerhans rule out pancreatic adenocarcinoma for what?

Answer 81

chronic pancreatitis

Question 82

45 yo female experiencing pain and fullness of the abdomen with compression of organs and nearby tissues. CT shows a large solitary 9 cm mass with thick walls on the tail (or body) of the pancreas. Biopsy reveals tall columnar mucin epithelium with cellular atypia and ovarian-type stroma* (scant cytoplasm, uniform elongated wavy nuclei)

Answer 82

mucinous cystic neoplasm

Question 83

66-year-old diabetic notes intermittent epigastric pain, anorexia, back pain, and weight loss for the last year. CT reveals a neoplasm within the main pancreatic duct located at the pancreatic head. The endoscopy revealed mucin extravasation at the ampulla of the vater and the biopsy showed papillary mucin-producing epithelial cells in the main and large duct of the pancreas.

Answer 83

intraductal papillary mucinous neoplasm (IPMN)

Question 84

Upon a routine physical, a large >4cm abdominal mass was noted on an asymptomatic 55-year-old that was discrete and well demarcated. A biopsy of the pancreatic tail revealed a sponge-like honeycomb appearance filled with serous fluid and numerous tightly packed cells with no communication to the pancreatic duct system.

Answer 84

serous cystic neoplasm

Question 85

What is a likely precursor to pancreatic ductal adenocarcinoma and characterized by noninvasive low to high-grade nuclear atypia involving pancreatic ducts <5mm diameter?

Answer 85

pancreatic intraepithelial neoplasia (PanIN)

Question 86

What form of PanIN includes marked nuclear atypia, hyperchromatic nuclei, loss of polarity, cribiforming, and luminal necrosis?

Answer 86

high grade: type 3 + carcinoma in situ

Question 87

Inactivation of what genes tend to be associated with PanIN-3?

Answer 87

TP53, SMAD4, BRCA2

Question 88

What makes up nearly 90% of all pancreatic neoplasms: characterized by malignant epithelial neoplasm from the ductal system with glandular differentiation?

Answer 88

pancreatic exocrine neoplasia: infiltrating ductal adenocarcinomas

Question 89

70 yo AA male with Peutz Jeghers Syndrome comes in painless jaundice ad pruritus, weight loss, epigastric pain that radiates to teh back, anorexia, and depression. Serum CA19-9 and Lewis blood antigen are elevated. A CT shows a solid ill-defined mass on the pancreatic head obstructing the common bile duct alongside metastasis of the lungs and liver. Additional complications including Courvoisier's gallbladder and migratory thrombophlebitis (trousseau syndrome). A biopsy reveals loss of lobular configuration and sharply angulated glands near thick-walled vessels.

Answer 89

pancreatic adenocarcinoma (infiltrating ductal)