Neoplasms of the GB, Liver, Pancreas Flashcards
What is the gross appearance of Focal nodular hyperplasia?
Well-demarcated
central, depressed stellate scar
What demographic (age, gender) is focal nodular hyperplasia most common??
F>M, 30-50 yo
What liver pathology is Focal nodular hyperplasia a/w?
vascular lesions of liver (hyperperfused)
What is the most common benign neoplasm of the liver? what is the gross appearance?
Cavernous hemangiomas
soft red-blue subcapsular nodule (<2cm)
mistaken for metastasis (on imaging, surgery)
What are the risks for developing Hepatocellular adenoma?
oral contraceptives/anabolic steroids
cessation can lead to complete regression
What is the gross appearance of hepatocellular ADENOMA?
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
What are the 3 subtypes of Hepatocellular adenoma?
- HNF1-a inactivation
- B-catenin activation
- Inflammatory
Stratify the malignancy risk of HNF1-a inactivation, B-catenin activation, and Inflammatory Hepatocellular adenoma.
- B-catenin activation!!!!!!!! >
- Inflammatory >
- HNF1-a inactivation
What are characteristics of HNF1-a inactivation hepatocellular adenomas?
fatty liver nodules
a/w MODY-3
least malignancy risk
What are characteristics of Inflammatory Hepatocellular adenomas?
JAK/STATE pathway mutations
–mutations in GP130 (coreceptor for IL-6)
small risk of malignant transformation
What is the difference between Large cell change and Small cell change as precursors to Hepatocellular Carcinoma?
Large Cell:
- normal N:C ratio
- near portal tracts
Small Cell:
- higher N:C ratio
- small expansive nodules
What are characteristics of High-grade dysplastic precursors to HCC?
cytologic/architectural atypia
(pseudoglands, trabecular thickening)
greater hepatic arterial blood supply
What is the most common PRIMARY liver malignancy?
what are rates of occurrence correlated with?
Hepatocellular carcinoma (HCC) -occurs in contest of cirrhosis
Risks:
Male
Countries with high rates of HBV, HCV or AFLATOXIN (Aspergillus mycotoxin)
What are 3 histopathologic features of HCC?
- cytologic atypia, increased N:C ratio
- Thickened hepatocyte trabeculae (>2 cells)
- Pseudoacini (pseudoglands) - hemorrhage/necrosis, lack portal areas
What are 3 pathogenic causes of HCC from chronic liver disease?
- HBC, HCV/aflatoxin/EtOH - synergistic damage
- TP53 mutations - MOST COMMON early event
- IL-6/JAK/STAT
What are clinical features and radiology of HCC?
clinical features:
abdominal pain, malaise, weight loss, jaundice
radiology
U/S: nodules
CT/MRI w/ contrast - increased arterialization
What is the prognosis of HCC? how does it metastasize?
poor prognosis
Metastasis: via hepatic venous system (to lung)
What is the demographic of Fibrolamellar variant of HCC? what is the prognosis?
younger (<35)
favorable prognosis
What is the most common liver tumor in children (<3 yo)? what is the tumor histopathology?
Hepatoblastoma
histo: primitive, fetal-type tissue
what is the gene mutation of hepatoblastoma?
APC (Wnt signalling pathway)
What is a cholangiocarcinoma? What are risks?
tumor of bile duct epithelium
-90% Extrahepatic (Klatskin tumor: perihilar)
Risks: Liver fluke Inflammatory: PSC, PBC, hepatolithiasis, fibrocystic HBV, HCV NAFLD
What is the the clinical presentation and prognosis of Cholangiocarinoma?
Sx: Abd pain, weightloss
JAUNDICE - Conjugated bilirubin predominant (obstructive)
Older pts
RARELY presents with cirrhosis
Prognosis: dismal
What is the most common form of Gallbladder Neoplasms? What is the epidemiology, and risk factors?
Adenocarcinomas
Epidem: Native American, Hispanic
F>M
Risks: chronic inflammation (CHOLELITHIASIS)
What is the molecular pathogenesis of Gallbladder Carcinomas? what is Tx?
Overespression of ERBB2 (Her-2/neu) receptor
Tx: Trastuzumab
What accounts for 75% of pancreatic cysts? what are some characteristics of it?
Pancreatic Pseudocysts
- Lack epithelial lining (from constant destruction/repair: pancreatitis)
- Cyst fluid high in LIPASE, AMYLASE
What are 3 types of Benign cystic neoplasms of pancreas?
- Serous cystic neoplasms
- Mucinous cystic neoplasms
- Intraductal papillar mucinous neoplasms (IPMN)
What are some characteristics of Serous cystic neoplasms of pancreas?
- uniformly benign
- multicystic (grossly: Spongy)
- Tail of pancreas
- VHL gene mutation
What are some characteristics of Mucinous cystic neoplasms of pancreas?
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)
What are some characteristics of Intraductal Papillary Mucinous Neoplasms (IPMN) of pancreas?
- M>F
- Head of Pancreas > tail
- arise within large pancreatic ducts
- Can progress to invasive cancer
- KRAS, TP53 mutations
What are is the pathogenesis of Pancreatic intraepithelial neoplasia (PanIn) => PANCREATIC CANCER
progressive telomeric shortening
KRAS, TP53 mutations
model for progression: PanIn I, II, III
Where is pancreatic cancer most commonly located?
60% in head
distant metastasis to liver and lung
What is the clinical Sx of pancreatic Cancer?
typically “silent” before metastasis
Sx: “Painless Jaundice (obstructive) w weightloss)
Trousseau sign: migratory thrombophlebitis
Labs: serum CA 19-9, CEA
