Primary adenocarcinoma of the duodenum associated with cutaneous neurofibromatosis (von Recklinghausen’s disease)

McGlinchey, J. J.; Santer, G. J.; Haqqani, M T

doi:10.1136/pgmj.58.676.115

Cited by 23 publications

(10 citation statements)

References 10 publications

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“…These tumors were located in the ampulla of Vater in five cases (13, 18 -20, and the present one), in the head of the pancreas in four cases (16,(21)(22)(23), in the duodenum in four cases (10,19,22,24), in the bile duct in one case (23), and in the gallbladder in one case (13). From this analysis it emerges that the association of epithelial cancers with NF-1 is equally distributed between the sexes (seven males, five females, and two of unknown sex), in accordance with the general distribution of NF-1.…”

Section: Discussionmentioning

confidence: 99%

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Ampullary Adenocarcinoma in Neurofibromatosis Type 1. Case Report and Literature Review

et al. 2001

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Periampullary tumors in patients affected by Neurofibromatosis Type 1 (NF-1) are usually carcinoids or stromal tumors and, rarely, adenocarcinomas. We report a case of an adenocarcinoma of the ampulla of Vater in a 54-year-old woman with NF-1 admitted to the hospital with jaundice and undergoing pancreato-duodenectomy. Histologically, the resected specimen showed an adenocarcinoma of the ampulla as being a part of a complex atypical epithelial proliferation extended from the papilla to the mucosa of the duodenum and distal choledochus, islet-cell adenomatosis of the pancreas and multiple gastric, duodenal, jejunal stromal tumors. The ampullary and periampullary adenocarcinomas in NF-1 patients have peculiar features, suggesting a widespread predisposition to cancer development in periampullary tissues and requiring widely demolitive surgery. Moreover, they occur at a younger age than those occurring in non-NF-1 patients, may be associated with additional periampullary epithelial tumors, are often operable and may present long survival.

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Section: Discussionmentioning

confidence: 99%

“…4). The association of an adenocarcinoma of the papilla with dysplasia of the duodenum and of the terminal choledochus (24) and an adenocarcinoma of the pancreas with focal atypical proliferation of the pancreatic ducts (16) have already been described.…”

Section: Discussionmentioning

confidence: 99%

Ampullary Adenocarcinoma in Neurofibromatosis Type 1. Case Report and Literature Review

et al. 2001

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“…The coexistence of neurofibromatosis with small-bowel adenocarcinoma is exceedingly rare (Table 1) (Albores-Saavedra et al 1974;Ho et al 1980;McGunchey et al 1982;Nelson 1982;Jones et al 1987;Benharroch et al 1992;Joo et al 2002). A review of the English-language literature disclosed seven reported cases of adenocarcinoma of the small bowel in patients with neurofibromatosis.…”

Section: Discussionmentioning

confidence: 99%

Intestinal neurofibromatosis and small-bowel adenocarcinoma: a single case study

Stratopoulos

Papakonstantinou

Anagnostopoulos

et al. 2009

European Journal of Cancer Care

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Patients with Von Recklinghausen's disease (neurofibromatosis type 1) are at increased risk of developing various tumours. However, the coexistence of neurofibromatosis with small-bowel adenocarcinoma is exceedingly rare. We present an uncommon case of neurofibromatosis type 1, involving the small bowel in a 73-year-old man, who was admitted to our department with signs of acute abdomen. At laparotomy, multiple mesenteric and intramural nodules were seen in the distal ileum. These nodules obstructed ileal lumen, while the intestine wall was perforated in one point. A wide resection of the affected ileum together with all visible nodules in the adjacent mesentery was performed. Histology revealed neurofibromatosis type 1 with malignant transformation to small-bowel adenocarcinoma. The patient had no additional therapy. In a follow-up of 2 years, the patient is very well and there was no recurrence of the disease. We suggest that adenocarcinoma of small bowel should be considered in the evaluation of acute abdominal pain in neurofibromatosis patients.

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“…7 Subsequently, five patients have been reported with neurofibromatosis and smallbowel adenocarcinoma. [8][9][10][11][12] We report a case of primary duodenal adenocarcinoma in a patient with neurofibromatosis type 1, and review the literature pertaining to this condition.…”

Section: Introductionmentioning

confidence: 99%

Primary duodenal adenocarcinoma associated with neurofibromatosis type 1

et al. 2002

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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.

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Primary adenocarcinoma of the duodenum associated with cutaneous neurofibromatosis (von Recklinghausen’s disease)

Abstract: A case of primary adenocarcinoma of the duodenum in a 29-year-old man with neurofibromatosis (von Recklinghausen’s disease) is described. As far as can be ascertained, such an association has not been described before.

Cited by 23 publications

References 10 publications

Ampullary Adenocarcinoma in Neurofibromatosis Type 1. Case Report and Literature Review

Ampullary Adenocarcinoma in Neurofibromatosis Type 1. Case Report and Literature Review

Intestinal neurofibromatosis and small-bowel adenocarcinoma: a single case study

Primary duodenal adenocarcinoma associated with neurofibromatosis type 1

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