2002
DOI: 10.1007/s005350200024
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Primary duodenal adenocarcinoma associated with neurofibromatosis type 1

Abstract: 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 sugg… Show more

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Cited by 15 publications
(13 citation statements)
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“…Taking into consideration the prevalence of these cancers in the general population, the association of adenocarcinomas in the colon, esophagus, stomach, biliary tract, and pancreas with NF1 may be incidental. On the other hand, the reports of adenocarcinomas of the small intestine in patients with NF1 are more difficult to dismiss as coincidental because of the number of reported cases for an uncommon neoplasm (13,70).…”
Section: Adenocarcinomamentioning
confidence: 95%
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“…Taking into consideration the prevalence of these cancers in the general population, the association of adenocarcinomas in the colon, esophagus, stomach, biliary tract, and pancreas with NF1 may be incidental. On the other hand, the reports of adenocarcinomas of the small intestine in patients with NF1 are more difficult to dismiss as coincidental because of the number of reported cases for an uncommon neoplasm (13,70).…”
Section: Adenocarcinomamentioning
confidence: 95%
“…The majority of the small intestinal adenocarcinomas occurring in patients with NF1 are peri- ampullary in location (68,70). Williams et al (76) remarked that the association between adenocarcinoma of the small intestine and NF1 should be considered tenuous because of the well-established association between carcinoids and NF1 and the difficulty in distinguishing between adenocarcinoma and carcinoid histopathologically.…”
Section: Adenocarcinomamentioning
confidence: 97%
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“…However, the increased prevalence of these tumors in the general population compared with NF1 patients suggests an incidental finding. Having said that, adenocarcinoma of the small intestine in patients with NF1 may be an exception given the large number of reported cases among NF1 patients [214]. The majority of small intestinal adenocarcinoma occurring in patients with NF1 are periampullary in location and this was discussed previously [212,214].…”
Section: Additional Nf1-associated Malignanciesmentioning
confidence: 98%
“…[2][3][4] There also have been seven case reports in the literature that indicate an association between neurofibromatosis and small-bowel adenocarcinoma. 5 It has been suggested that there is an association with adenocarcinoma of the stomach, 6 oesophagus, 7 and pancreas. 8 Neurofibromas consist of proliferated Schwann cells and fibroblasts in a collagen matrix surrounding nerve fibers.…”
Section: Discussionmentioning
confidence: 99%