PubMed · 6115206
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1981-08-29. Endoscopic sphincterotomy.. https://pubmed.ncbi.nlm.nih.gov/6115206/
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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.
Papilla Vateri carcinoid is among the very rarely occurring locations of the lesion. The small lumen of the papilla is the underlying cause of becoming clinically manifest with icterus rather early. Accessibility of the process for endoscopic biopsy facilitates the diagnosis. Even histologically it is very difficult to differentiate benign from malignantly altered carcinoids. Only the existence of metastases confirm with certainty their malignant nature. An observation on papilla Vateri carcinoid in a 60-year-old woman, proved preoperatively through cytological study, is reported. Intraoperative--no metastases are discovered. Sparing pancreatoduodenal resection is performed with a good functional outcome, recorded at the checkup examination after two years.
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