Malignant neoplasms of biliary tract Flashcards
A malignant tumor arising from the bile ducts within and outside of the liver.
Cholangio-Carcinoma (CCA)
What are the risk factors for developing Cholangio-Carcinoma (CCA)?
Primary Sclerosing Cholangitis.
Choledochal cysts.
HCV infection.
Previous exposure to Thorotrast.
How is Cholangio-Carcinoma (CCA) classified?
Intra-hepatic forms.
Extra-hepatic forms (80-90% of cases):
Peri-hilar tumors (Klatskin tumors): Located at the confluence of the right and left hepatic ducts.
Distal bile duct tumors.
What are the characteristics of Klatskin tumors?
Slower growth compared to other CCAs.
Prominent fibrosis.
Infrequent distal metastases.
Microscopic features of CCA
Mostly, Adeno-CAs with or without mucin
secretion
Abundant fibrous stroma
Macroscopic features of CCA
Size: Small lesions
Mostly: Firm, gray nodules within the bile
duct wall; Alternatively: Diffusely infiltrative,
papillary or polypoid lesions
Where do Intrahepatic CCAs typically occur?
In the non-cirrhotic liver.
What are the macroscopic features of Intrahepatic CCAs?
Tree-like tumorous mass along the intra-hepatic portal tract system, or
A massive tumor nodule.
Tumor substance is extremely firm and gritty.
Extensive metastases to:
Intra-hepatic regions.
Lymph nodes.
Lungs, bones, adrenals, and brain via haematogenous spread.
What are the microscopic findings in Intrahepatic CCAs?
Majority: Well to moderately differentiated sclerosing adenocarcinomas.
Presence of glandular and tubular structures lined by cuboidal to low columnar epithelial cells.
Marked desmoplasia: Dense collagenous stroma separating glandular elements.
Occasional mixed variants with elements of both Hepatocellular Carcinoma (HCC) and CCA.
What is the most common risk factor for gallbladder carcinoma?
Cholelithiasis; gallstones are present in 95% of cases.
What is the epidemiology of gallbladder carcinoma?
Most common malignancy of the extra-hepatic biliary tract.
Frequently occurs in the seventh decade of life.
Mean 5-year survival rate: About 5-12%.
Only 0.5% of patients with cholelithiasis develop gallbladder cancer.
What are the growth patterns of gallbladder carcinoma?
Infiltrating (more common):
“Scirrhous” tumors: Very firm consistency.
Poorly defined diffuse thickening and induration of the gallbladder wall.
May cause deep ulceration and fistula formation to adjacent viscera.
Exophytic:
Grows as an irregular cauliflower mass with invasion of the underlying wall.
Luminal portion: Necrotic, hemorrhagic, and ulcerated.
What are the common sites of gallbladder carcinoma?
Fundus and neck of the gallbladder.
What is the most common histological type of gallbladder carcinoma?
Adenocarcinomas, with some showing:
Papillary architecture (well/moderately differentiated, better prognosis).
Infiltrative growth pattern (poorly differentiated to undifferentiated).
What are the clinical symptoms of gallbladder carcinoma?
Symptoms are insidious and similar to cholelithiasis:
Abdominal pain.
Jaundice.
Anorexia.
Nausea and vomiting.
