PubMed · 7182333
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Z Halpern, E Barden, S Bar-Meir. 1982-11-15. [Papillary dysfunction].. https://pubmed.ncbi.nlm.nih.gov/7182333/
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The localization of the carcinoid tumors of the gastrointestinal tract--as a part of the APUD system--is extremely seldom at the region of ampulla of Vater. A case of congenital neurofibromatosis is reported in a 49-year-old male patient, with a carcinoid tumor of the ampulla of Vater in the background of long-standing, atypical biliary complaints. Periampullary neoplasm of neural-crest origin are very rare, up to this time 73 cases of Vater papilla carcinoid tumors were described. Regarding the development of VP carcinoid in neurofibromatosis, our patient is the 21st reported case in the world literature, and the first one in Hungary. The authors review the literature, giving special attention to the risk of periampullary neoplasms in von Recklinghausen's disease, the need of accurate diagnosis and the correctly selected operative intervention.
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Forty-five extrahepatic bile duct carcinomas (i.e., tumors of the region proximal to the duct junction, including Klatskin tumors, tumors of the lower mid-region, and tumors of the ampulla of Vater) and 11 gallbladder carcinomas were immunohistochemically examined for p53 protein expression, using the DO-7 monoclonal (mAb) and the CM-1 polyvalent (pAb) antibodies and an antigen retrieval method. Because the DO-7 mAb was found to be significantly more reliable than the CM-1 pAb in detecting p53 immunoreactivity, the immunohistochemical results obtained with the former antibody were used for comparing p53 protein immunoreactivity with tumor site, tumor grade, and survival of patients. Approximately one third (3 of 10) of the proximal tumors were found to express weak p53 immunopositivity, whereas moderate immunopositivity and higher rate (18 of 29) was observed in tumors of the lower mid-region. Finally, moderate and marked p53 immunopositivity was observed in tumors of the ampulla (5 of 6) and gallbladder (8 of 11). In tumors of the lower mid-region of the ampulla and the gallbladder, a significantly higher p53 positivity was noted in high-grade compared with low-grade neoplasms. For the cases in which complete follow-up was available (11 tumors of the low mid-region), median survival of patients with p53-negative tumors was 25.7 months, whereas survival of those with p53-positive tumors was 5.2 months.(ABSTRACT TRUNCATED AT 250 WORDS)