Italy
Case Report
Primary Ampullary Adenocarcinoma and Von Recklinghausen’s Disease: A Rare Association
Author(s): Taddei A, Messerini L, Papi L, Castiglione F, Ringressi MN and Bechi PTaddei A, Messerini L, Papi L, Castiglione F, Ringressi MN and Bechi P
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 m.. Read More»
DOI:
10.4172/2165-7920.1000760
Journal of Clinical Case Reports received 1345 citations as per Google Scholar report