Primary duodenal adenocarcinoma associated with neurofibromatosis type 1.
It is well known that patients with neurofibromatosis have an increased incidence of various neoplasms, most of these being tumors of neural-crest origin, including neurofibromas, leiomyomas, ganglioneuromas, paragangliomas, and carcinoids. However, the occurrence of small-bowel adenocarcinoma is rare. In this article, we report a patient with small-bowel adenocarcinoma, the seventh such reported case. We include a review of the literature and a brief discussion of the implications of this association. We suggest that the association between small-bowel adenocarcinomas and neurofibromatosis may not be fortuitous, and that small-bowel adenocarcinoma should be considered, in particular. in the differential diagnosis of gastrointestinal symptoms in patients with neurofibromatosis. Also, a definite histologic diagnosis must be made, with the employment of special staining techniques, because of the differing prognoses for various cell types.
Joo
Young-Eun
YE
Department of Internal Medicine, Chonnam National University Medical School, Kwangju, Korea.
Kim
Hyun-Soo
HS
Choi
Sung-kyu
SK
Rew
Jong-Sun
JS
Park
Chang-Soo
CS
Kim
Sei-Jong
SJ
D000230:Adenocarcinoma / Q000150:complications / Q000175:diagnosis*; D001706:Biopsy; D002760:Cholangiopancreatography, Endoscopic Retrograde / Q000379:methods; D004379:Duodenal Neoplasms / Q000150:complications / Q000175:diagnosis*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications / Q000175:diagnosis*