Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0268596 (EMA)
 2,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Although clear cell carcinomas have been described in numerous anatomic sites, their occurrence in the gallbladder and extrahepatic bile ducts (EHBD) is practically unknown. We report 10 such cases. Seven arose in the gallbladder and three in the EHBD; all patients with gallbladder tumors were females with cholelithiasis whose ages ranged from 56 to 68 years. Patients with EHBD tumors were younger (38 and 40 years of age) and had extrahepatic biliary obstruction and abdominal pain. Two patients with gallbladder carcinomas had elevated serum carcinoembryonic antigen (CEA) levels, and another without hepatic involvement had markedly elevated circulating levels of alpha-fetoprotein (AFP). Histologically, nine tumors were adenocarcinomas and one was a squamous cell carcinoma. Seven adenocarcinomas consisted of cords, sheets, nests, papillae, and trabeculae of clear cells with well-defined cytoplasmic borders. Two were composed predominantly of glands and papillary structures. The cells contained PAS-positive diastase-labile granules and were cytokeratin- and EMA-positive and immunoreactive for erythropoiesis-associated antigen. One gallbladder tumor contained areas of hepatoid differentiation, a feature described in gallbladder neoplasms only once before. These areas were AFP-positive and immunoreactive for CEA. By electron microscopy, they showed hepatoid differentiation with formation of bile canaliculi. In two gallbladder tumors, neoplastic cells contained subnuclear vacuoles reminiscent of early secretory endometrium. Foci of conventional adenocarcinoma or mucinous carcinoma were recognized in all nine tumors. The squamous cell carcinoma showed only foci of squamous differentiation with keratinization. The clear cells of this neoplasm had a trabecular and solid growth pattern. These clear cell neoplasms of the gallbladder and EHBD must be differentiated from metastatic renal cell carcinoma, based upon the presence of areas of conventional adenocarcinoma or foci of squamous differentiation since results of special stains and immunohistochemistry are similar in both neoplasms. One of the patients with EHBD carcinoma is alive and symptom-free 6 years following right hepatic lobectomy. Five patients with gallbladder tumors had direct extension into the liver and died with metastases. Two are living with metastases.
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PMID:Clear cell carcinomas of the gallbladder and extrahepatic bile ducts. 780 41

We report four previously undescribed primary giant cell tumors of the extrahepatic biliary tree and morphologically compare them with 10 anaplastic spindle and giant cell carcinomas with osteoclast-like giant cells of the gallbladder. Two giant cell tumors were located in the distal common bile duct; one in the cystic duct and one in the gallbladder. The 3 patients with bile duct tumors were male, and the only patient with a gallbladder tumor was a female. The age of the patients ranged from 45 to 60 years with an average of 55 years. The patients with bile duct tumors presented with biliary obstruction, and the patient with a gallbladder tumor presented with symptoms of cholelithiasis and a gallbladder mass. Histologically, the tumors were similar to giant cell tumors of bone. They consisted of a mixture of mononuclear and multinucleated osteoclast-like giant cells. The mononuclear cells showed no atypical features, and their nuclei were similar to those of the multinucleated giant cells. CD163 immunoreactivity was restricted to the mononuclear cells, whereas CD68 and HAM 56 labeled only the multinucleated osteoclast-like giant cells. The mononuclear cells were EMA-positive but did not express cytokeratins. Follow-up showed that 3 patients were alive and disease-free 3.7 to 7 years after surgery. The anaplastic spindle and giant cell carcinomas contained a fewer number of osteoclast-like giant cells, and their mononuclear cells showed considerable variation in size and shape, marked cytologic atypia, and numerous mitotic figures. They were focally cytokeratin positive (AE1/AE3; CAM 5.2) and did not label with CD163, CD68, and HAM 56. The benign osteoclast-like giant cells showed immunoreactivity for CD68 and HAM 56 but were negative for CD163 and cytokeratins. Giant cell tumors of the extrahepatic biliary tree are benign true histiocytic neoplasms that should be distinguished from the highly lethal anaplastic spindle and giant cell carcinomas with osteoclast-like giant cells by detailed cytologic analysis and immunohistochemical stains for CD163, CD68, HAM 56, and cytokeratins.
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PMID:Giant cell tumor of the extrahepatic biliary tree: a clinicopathologic study of 4 cases and comparison with anaplastic spindle and giant cell carcinoma with osteoclast-like giant cells. 1822 38