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Case Report Open Access
Objective: Neurofibromatosis type 1 (NF1) is an autosomal dominant inherited disease. Although only a minority
of NF1 patients develops malignancy as a complication of the disorder, cancer (mainly represented by tumors
involving the nervous system) remains an important cause of morbidity and mortality. A case of the rare association
between adenocarcinoma of the Vater ampulla and NF-1 is here reported. The patient accepted Whipple operation.
Histologic analysis of the whole surgical specimen revealed an adenocarcinoma limited to the ampulla of Vater,
without lymphonodal involvement.
Methods: A study of the possible presence of genetic alterations, which could demonstrate a molecular correlation
between NF-1 and periampullary epithelial neoplasms, was performed using the intragenic NF1 microsatellites.
Results: Adenocarcinoma sample retained heterozygosity for the informative microsatellites; furthermore,
microsatellite analysis was unable to detect any LOH involving the NF1 gene.
Conclusion: Our results suggest that Ampulla’s adenocarcinomas are an occasional event in NF1, accordingly
to the epithelial but not neuroectodermical origin of this tumour.
Ampullary adenocarcinoma, NF1, Vater ampulla, Pancreatoduodenectomy, von Recklinghausenâs disease, Medical Case Reports