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Chirurgia 2003 October-December;16(5-6):183-4

language: Italian

Adenocarcinoma of Vater's ampulla and ileal stromal tumor in neurofibromatosis type I. Case report and literature review

Di Lecce F., Bonfatti Paini P., D'Errico G., Pagliari C., Malchiodi G., Sabag R., Abruzzi L., Pampari G.


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Neurofibromatosis type 1 (NF1) or von Recklinghausen's disease is an autosomal dominant disorder that represents a major risk factor for development of benign and malignant tumours. A case of adenocarcinoma of Vater's ampulla and ileal stromal tumour, treated with pancreato-duodenectomy and ileal resection, in a 37-year-old man with von Recklinghausen's disease is reported. The relevant literature is reviewed with special attention to the risk of periampullary neoplasms in NF1 and the correct approach to the diagnosis and treatment of this rare association is discussed.

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