Week 5: hepatic and biliary tumors Flashcards
Liver cell adenoma-clinical
- uncommon, usually single mass
- associated with estrogen usage (oral contraceptives), androgen usage, DM, spontaneous
- women, 20-39, present with abdominal mass, or pain secondary to hemorrhage
- normal aminotranferases, normal alk phos, normal alpha-fetoprotein
- Rx: discontinue OCP, surgical removal to prevent risk of necrosis
- rarely progresses to hepatocellular carinoma
Liver cell adenoma-pathology
GROSS
-usually solitary, well circumscribed, may be encapsulated. yellow-brown. May have focal necrosis and hemorrhage
MICRO
-uniform slightly hydraulic cells. Liver cords are 1-2 cell thick. May have fatty change, cholestasis.
Focal nodular hyperplasia-clinical
- hyperplastic, not neoplastic, benign hepatocytes
- 7% of benign primary hepatic tumors
- mostly women, 20-40 yo, often found incidentally
- normal liver tests and normal AFP
- pathogenesis: abnormal central artery with liver cell damage and adjacent hyper plastic response of hepatic parenchyma. From repeated vascular thrombosis, but unknown.
- Rx: conservative. no evidence of malignant transformation.
Focal nodular hyperplasia-pathology
GROSS
-solitary lesions, non encapsulated, classic central radiating scar. many peripheral, smaller nodules subdivided by fibrous septa.
MICRO
-central radiating fibrous septum, lymphocytic infiltration, increased vascular channels, atypical bile duct elements at periphery. Cords 1-2 cells thick.
Cavernous Hemangioma- clinical
- single or multiple benign vascular lesions with communicating vascular spaces
- most common benign tumor
- more so women, mean age 46
- mostly asymptomatic. Symptoms: ab swelling, RUQ mass, pain,
- Dx: imaging
- Rx: conservative. Resection is an option when symptomatic. Hepatic artery embolization.
Cavernous hemangioma -pathology
GROSS
-hemorrhage
MICRO
-numerous congested vascular spaces around central fibrous tissue. Communicating vascular spaces, lined by single layer endothelial cells. Stroma composed of collagen. Can have fibrosis and calcification.
Hepatocellular carcinoma- clinical
- most common primary hepatic tumor in adults, 3/4 of all neoplasms
- associated with underlying chronic liver disease (cirrhosis)
- composed of malignant hepatocytes, forming thick cords, acini and sheets
- most common etiology in Western countries is alcohol, in developing countries is HBV
- Rx: surgical resection with or without chemotherapy.
Etiologic factors and HCC
- HBV
- DNA virus that incorporates into host genome. 200x increase risk for HCC. Other factors play a role. - HCV
- cirrhosis usually present when HCC develops, though not always. 3-4x more likely to develop HCC with HCV. - Aflatoxin B1
- Aspergillus flavus. Common contaminant of food that has been stored extensively in hot/humid environments. - Alcoholic cirrhosis
- direct mutagenic effects of EtOH and acetaldehyde. Cyclic process of liver cell damage and regeneration. - Hemochromatosis
- oncogenic potential of Fe deposition. - Drugs and toxins
- estrogen, vinyl chloride, thorotrast,
Alpha fetoprotein and HCC
- most useful biochemical marker for HCC
- alpha 1 globulin produced normally by fetal tissue, but drops off by 1 yo.
- can be elevated in inflammatory conditions of the liver.
- in setting of chronic liver disease patient developing hepatic mass, alpha fetoprotein elevation greater than 400 ng/ml indicates HCC
Clinical presentation of HCC
- mostly epigastric discomfort or fullness
- ascites, vatical bleed
- can be found incidentally
- should be suspected in an alcoholic cirrhotic who has stopped drinking for a long period and suddenly develops severe liver failure
HCC pathology
GROSS
-tumor appears most often in cirrhotics hemorrhage within larger nodules. multifocal. May have necrosis.
MICRO
-hepatic cords are thicker (put to 10 cells thick), variable trabecular structures. increased nuclear/cytoplasmic ratio. inclusions, fat, mallory bodies, and bile may be present in cytoplasm.
Fibrolamellar hepatocellular carcinoma- clinical
- 14-40% of primary hepatic malignancies in < 35 yo
- single mass lesion, usually in non-cirrhotic livers.
- slow growing tumors with septet fibrosis.
- liver tests are non diagnostic, AFP elevated in 10%.
- Rx: surgical resection. 56% 5 year survival, better than trabecular HCC.
Fibrolamellar HCC -pathology
MICRO
- fibrous “lamellar” bands of hypo cellular collagen
- thickened tumor cell cords
- pale body inclusions
Cholangiocarcinoma - clinical
- adenocarcinoma that arises from intrahepatic and extra hepatic bile ducts. Glands or tubules growing within a moderate or abundant fibrous stroma.
- mostly men, median age 63
- Clinical signs: ab pain, ascites, edema, jaundice, palpable mass
- labs: non diagnostic. Alk phos is elevated. AFP normal. CEA and CA19-9 elevated.
- Rx: resection, but most are non resectable. Poor prognosis.
Etiology of cholangiocarcinoma
ETIOLOGY -associations
- Fibrocystic disease: polycystic dz, congenital hepatic fibrosis, Caroli’s dz.
- Inflammatory bowel dz: Chronic ulcerative colitis
- recurrent pyogenic cholangiohepatitis
- Infection: Clonorchus sinensis
- Drugs: thorotrast
