Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P04637 (p53)
 77,613 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a case of biliary adenofibroma in a 47-year-old woman, who presented with right upper quadrant pain for several months. Abdominal imaging revealed a 16-cm solid and cystic mass in the left hepatic lobe. Histologically, the tumor showed two distinct components: 1) cystic and tubular structures lined by low columnar to cuboidal biliary-type epithelium, and 2) a dense fibrous stroma composed of spindle-shaped cells with only mild nuclear pleomorphism and inconspicuous nucleoli. Mitoses and stromal invasion were absent. The glandular epithelium stained positively for keratin AE.3/Cam 5.2, cytokeratin 7, cytokeratin 19, carcinoembryonic antigen, and epithelial membrane antigen and had a low Ki-67 proliferative index. In addition, the epithelium was positive for D10 but did not stain for 1F6 or acid mucin with alcian blue stain. This staining pattern, similar to bile duct hamartoma (von Meyenburg complex) with which this tumor shares morphologic similarity, suggests that biliary adenofibroma originates from interlobular or larger bile ducts. Three years after a subtotal resection no metastasis or significant tumor growth was noted. However, given the marked nuclear p53 immunoreactivity and tetraploidy status observed in this tumor, we cannot exclude that biliary adenofibroma may represent a premalignant process that warrants complete resection and thorough histopathologic examination.
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PMID:Biliary adenofibroma: a rare neoplasm of bile duct origin with an indolent behavior. 1271 55

Rokitansky-Aschoff sinuses (R-As) are epithelial invaginations that extend down the gallbladder wall through the smooth muscle gaps and by this pathway they reach the subserosal connective tissue. We report here 5 cases of mucin-containing R-As that ruptured, and as a result, extracellular mucin escaped into the adjacent stroma. They were compared with 10 mucinous carcinomas of the gallbladder. Three cases of mucin-containing R-As accompanied by abundant extracellular mucin deposits with epithelial strips, glands and papillary structures were misinterpreted as mucinous carcinomas. Because of this, the patients were subjected to a second unnecessary radical surgical procedure. In the remaining 2 cases, the extracellular mucin associated with R-As was acellular and regarded as focal. Three patients were men and 2 women whose ages ranged from 2 to 71 years (average age 52 y). The youngest patient was a 2-year-old boy in whom the mucin-containing R-As were discovered incidentally by magnetic resonance imaging. He was later diagnosed with metachromatic leukodystrophy. The remaining 4 patients complained of right upper quadrant pain and had a thickened gallbladder wall identified by computed tomography and magnetic resonance imaging. There were gallstones in 3 patients. The R-As located in the lamina propria contained mucin but were not cystically dilated whereas those located in the subserosa were mucin-filled and often cystically dilated. Detached fragments of biliary epithelium, small glands, and papillary structures lacking cytologic atypia and mitotic figures were identified in the abundant mucin deposits located in the subserosa of 3 cases. The overlying surface gallbladder epithelium exhibited papillary hyperplasia with focal intestinal metaplasia in 2 patients, one of which had metachromatic leukodystrophy. The lack of reactivity for carcinoembryonic antigen and p53 and the low proliferative activity as measured by MIB-1 labeling index provided additional support to the benign nature of the lesion. Clues to separate mucin-containing R-As from mucinous carcinoma are provided. All 5 patients with mucin-containing R-As are disease free 8 months to 7 years after surgery (median follow-up of 39 mo).
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PMID:Mucin-containing Rokitansky-Aschoff sinuses with extracellular mucin deposits simulating mucinous carcinoma of the gallbladder. 2116