Neoplasms of the GB, Liver, Pancreas Flashcards

1
Q

What is the gross appearance of Focal nodular hyperplasia?

A

Well-demarcated

central, depressed stellate scar

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2
Q

What demographic (age, gender) is focal nodular hyperplasia most common??

A

F>M, 30-50 yo

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3
Q

What liver pathology is Focal nodular hyperplasia a/w?

A

vascular lesions of liver (hyperperfused)

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4
Q

What is the most common benign neoplasm of the liver? what is the gross appearance?

A

Cavernous hemangiomas
soft red-blue subcapsular nodule (<2cm)

mistaken for metastasis (on imaging, surgery)

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5
Q

What are the risks for developing Hepatocellular adenoma?

A

oral contraceptives/anabolic steroids

cessation can lead to complete regression

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6
Q

What is the gross appearance of hepatocellular ADENOMA?

A

well demarcated (encapsulated)
often:
1. hypovascular
2. large (>6 cm)
3. hemorrage + necrosis (Coagulative)
4. arranged in CORDS (trabeculae) <2 cell layers thick
—if cord is thicker than 2 cells = CARCINOMA

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7
Q

What are the 3 subtypes of Hepatocellular adenoma?

A
  1. HNF1-a inactivation
  2. B-catenin activation
  3. Inflammatory
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8
Q

Stratify the malignancy risk of HNF1-a inactivation, B-catenin activation, and Inflammatory Hepatocellular adenoma.

A
  1. B-catenin activation!!!!!!!! >
  2. Inflammatory >
  3. HNF1-a inactivation
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9
Q

What are characteristics of HNF1-a inactivation hepatocellular adenomas?

A

fatty liver nodules
a/w MODY-3
least malignancy risk

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10
Q

What are characteristics of Inflammatory Hepatocellular adenomas?

A

JAK/STATE pathway mutations
–mutations in GP130 (coreceptor for IL-6)
small risk of malignant transformation

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11
Q

What is the difference between Large cell change and Small cell change as precursors to Hepatocellular Carcinoma?

A

Large Cell:

  • normal N:C ratio
  • near portal tracts

Small Cell:

  • higher N:C ratio
  • small expansive nodules
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12
Q

What are characteristics of High-grade dysplastic precursors to HCC?

A

cytologic/architectural atypia
(pseudoglands, trabecular thickening)

greater hepatic arterial blood supply

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13
Q

What is the most common PRIMARY liver malignancy?

what are rates of occurrence correlated with?

A
Hepatocellular carcinoma (HCC)
-occurs in contest of cirrhosis

Risks:
Male
Countries with high rates of HBV, HCV or AFLATOXIN (Aspergillus mycotoxin)

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14
Q

What are 3 histopathologic features of HCC?

A
  1. cytologic atypia, increased N:C ratio
  2. Thickened hepatocyte trabeculae (>2 cells)
  3. Pseudoacini (pseudoglands) - hemorrhage/necrosis, lack portal areas
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15
Q

What are 3 pathogenic causes of HCC from chronic liver disease?

A
  1. HBC, HCV/aflatoxin/EtOH - synergistic damage
  2. TP53 mutations - MOST COMMON early event
  3. IL-6/JAK/STAT
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16
Q

What are clinical features and radiology of HCC?

A

clinical features:
abdominal pain, malaise, weight loss, jaundice

radiology
U/S: nodules
CT/MRI w/ contrast - increased arterialization

17
Q

What is the prognosis of HCC? how does it metastasize?

A

poor prognosis

Metastasis: via hepatic venous system (to lung)

18
Q

What is the demographic of Fibrolamellar variant of HCC? what is the prognosis?

A

younger (<35)

favorable prognosis

19
Q

What is the most common liver tumor in children (<3 yo)? what is the tumor histopathology?

A

Hepatoblastoma

histo: primitive, fetal-type tissue

20
Q

what is the gene mutation of hepatoblastoma?

A

APC (Wnt signalling pathway)

21
Q

What is a cholangiocarcinoma? What are risks?

A

tumor of bile duct epithelium
-90% Extrahepatic (Klatskin tumor: perihilar)

Risks:
Liver fluke
Inflammatory: PSC, PBC, hepatolithiasis, fibrocystic
HBV, HCV
NAFLD
22
Q

What is the the clinical presentation and prognosis of Cholangiocarinoma?

A

Sx: Abd pain, weightloss
JAUNDICE - Conjugated bilirubin predominant (obstructive)
Older pts
RARELY presents with cirrhosis

Prognosis: dismal

23
Q

What is the most common form of Gallbladder Neoplasms? What is the epidemiology, and risk factors?

A

Adenocarcinomas
Epidem: Native American, Hispanic
F>M

Risks: chronic inflammation (CHOLELITHIASIS)

24
Q

What is the molecular pathogenesis of Gallbladder Carcinomas? what is Tx?

A

Overespression of ERBB2 (Her-2/neu) receptor

Tx: Trastuzumab

25
Q

What accounts for 75% of pancreatic cysts? what are some characteristics of it?

A

Pancreatic Pseudocysts

  1. Lack epithelial lining (from constant destruction/repair: pancreatitis)
  2. Cyst fluid high in LIPASE, AMYLASE
26
Q

What are 3 types of Benign cystic neoplasms of pancreas?

A
  1. Serous cystic neoplasms
  2. Mucinous cystic neoplasms
  3. Intraductal papillar mucinous neoplasms (IPMN)
27
Q

What are some characteristics of Serous cystic neoplasms of pancreas?

A
  1. uniformly benign
  2. multicystic (grossly: Spongy)
  3. Tail of pancreas
  4. VHL gene mutation
28
Q

What are some characteristics of Mucinous cystic neoplasms of pancreas?

A
F>>M
precursor to malignant lesion
arise in TAIL
KRAS, TP53 mutations
Hist: thick, tenacious mucin, columnar cells w wall of dense stroma (similar to ovarian stroma)
29
Q

What are some characteristics of Intraductal Papillary Mucinous Neoplasms (IPMN) of pancreas?

A
  1. M>F
  2. Head of Pancreas > tail
  3. arise within large pancreatic ducts
  4. Can progress to invasive cancer
  5. KRAS, TP53 mutations
30
Q

What are is the pathogenesis of Pancreatic intraepithelial neoplasia (PanIn) => PANCREATIC CANCER

A

progressive telomeric shortening
KRAS, TP53 mutations
model for progression: PanIn I, II, III

31
Q

Where is pancreatic cancer most commonly located?

A

60% in head

distant metastasis to liver and lung

32
Q

What is the clinical Sx of pancreatic Cancer?

A

typically “silent” before metastasis
Sx: “Painless Jaundice (obstructive) w weightloss)
Trousseau sign: migratory thrombophlebitis

Labs: serum CA 19-9, CEA