USMLE – Tumors of the Gallbladder and Bile Duct
CARCINOMA OF THE GALLBLADDER
This is a rare tumor occurring more often in females and is usually encountered above the age of 70 years. More than 90% of such tumors are adenocarcinomas; the remainder are anaplastic or rarely, squamous tumors. Gallstones are usually present and are thought to be important in the etiology of the tumor.
The condition is usually diagnosed at surgery for gallstone disease. Occasionally it may manifest as repeated attacks of biliary pain and later persistent jaundice and weight loss. A gallbladder mass may be palpable in the right hypochondrium. Liver function tests show cholestasis, and gallbladder calcification (porcelain gallbladder) may be found on radiograph. The tumor may be diagnosed on ultrasonography. The treatment is surgical excision but local extension of the tumor beyond the wall of the gallbladder into the liver and surrounding tissues is not infrequent and palliative management is usually all that can be offered. Survival is generally short.
This uncommon tumor arises anywhere in the biliary tree from the small intrahepatic bile ducts to the papilla of Vater. The cause is unknown but it is associated with gallstones and primary sclerosing cholangitis. Primary sclerosing cholangitis is associated with ulcerative colitis, and cholangiocarcinoma may occur some years after proctocolectomy or as a presenting feature, with ulcerative colitis being discovered only subsequently.
The patient presents with jaundice which may be intermittent. Half the patients have upper abdominal pain and weight loss. The diagnosis is made by endoscopic cholangiography or percutaneous transhepatic cholangiography but can be difficult to confirm in patients with sclerosing cholangitis. Cholangiocarcinomas can occasionally be excised surgically, but most patients are treated by inserting drainage stents across the tumor using endoscopic or transhepatic techniques.
CARCINOMA AT THE PAPILLA OF VATER
Nearly 40% of all adenocarcinomas of the small intestine arise in relationship to the papilla of Vater and present with pain, anemia, vomiting and weight loss. Jaundice may be intermittent or persistent. Diagnosis is made by duodenal endoscopy and biopsy of the tumor. Ampullary carcinoma must be differentiated from carcinoma of the head of the pancreas and a cholangiocarcinoma because both these conditions have a worse prognosis.
Curative surgical treatment can be undertaken by pancreaticoduodenectomy or a segmental resection and the 5-year survival may be as high as 50%. When this is impossible a palliative bypass or insertion of a drainage stent is performed.
BENIGN GALLBLADDER TUMORS
These are uncommon, often asymptomatic and usually found incidentally at operation or autopsy. Cholesterol polyps, sometimes associated with cholesterolosis, papillomas and adenomas are the main types.