Neoplasms of Extrahepatic Bile Ducts
posted in The Aging Liver |Malignant neoplasms of the bile ducts are rare and often difficult to diagnose.
BENIGN NEOPLASMS
Papilloma and adenoma are the most common benign neoplasms of the bile ducts, although fibroadenoma, adenomyoma. leiomyoma, granular cell myoblastoma, neurinoma, and hamartoma also occur. Intermittent jaundice and right upper quadrant pain arc the most common symptoms. The treatment of choice is local excision.
MALIGNANT NEOPLASMS
By far the most common malignant neoplasm is adenocarcinoma. Bile duct cancer is more common in men: the average age at diagnosis is 60 yr. Tumors of the upper portion of the ducts (50% of all lesions) are intimately related to the liver; those of the middle portion, to the portal vein and hepalic artery; and those of the lower portion, to the pancreas and duodenum. Such localization hay both diagnostic and prognostic implications.
Predisposing factors include primary sclerosing cholangitis, Clo-norchis sinensis infestation, and industrial exposure (in automobile and rubber manufacturing plant workers).
Symptoms, Signs, and Diagnosis
Because of their location, malignant tumors usually cause early symploms. Jaundice occurs in almost all patients. Right upper quadrant pain occurs in > 50%. Other associated symploms and signs include weight loss, nausea, vomiting, anorexia, fever, chills, diarrhea, constipation, clay-colored stools, and hepatomegaly. If obstruction occurs below the cystic duel, a distended gallbladder (Courvoisier’s sign) may be palpaled.
Diagnosis*of large tumors is made by ultrasonography and CT scan. However, percutaneous transhepatic cholangiography or endoscopic retrograde cholangiopancreatography frequently is more importanl in making Ihe diagnosis and localizing the tumor. Liver function test re-sulls are consistent with cxlrahepatic obstruction.
Treatment
The prognosis for bile duct cancer is poor with a 5-yr survival rate of only 5%. Tumors in the proximal portion of the bile duct system rarely are operable. When they are, complex liver and duct resections are required, and there are few long-term survivors. Palliation may be accomplished by dilation and slent insertion either cndoscopically or via Iranshepatic cholangiography. Resectable tumors of the central portion of the bile ducts may be treated by local en bloc excision. Bile duct drainage is reestablished by hepaticojejunostomy.
For resectable tumors of the distal duets, radical resection and pancreatoduodenectomy (Whipple’s operation) provide some promising benefit wilh 5-yr survival rales of 20% to .30%. With nonresectable tumors, palliation may be accomplished by hepaticojejunoslomy or by stenl placement followed by internal radiation via endoscopic retrograde cholangiopancreatography or percutaneous catheter insertion into the duels. Chemotherapy, radiation therapy, and liver transplantation are ineffective.
