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Case Rep Gastrointest Med. 2012;2012:185730. doi: 10.1155/2012/185730. Epub 2012 Dec 4.

Goblet cell carcinoid in a patient with neurofibromatosis type 1: a rare combination.

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1
Department of Medicine, Hepatology and Gastroenterology, Aarhus University Hospital, Norrebrogade 44, 8000 Aarhus C, Denmark.

Abstract

Neuroendocrine tumors are rare tumors primarily located in the gastrointestinal tract. Goblet cell carcinoid is a rare subgroup of neuroendocrine tumors located in the appendix. Neurofibromatosis type 1 is an autosomal dominant disorder caused by a mutation in the NF1 gene. Patients with neurofibromatosis type 1 have an increased incidence of typical neuroendocrine tumors, but it is unknown if this is the case with goblet cell carcinoids. We describe a patient with both neurofibromatosis type 1 and goblet cell carcinoid, that according to literature would occur in 0.00017 per million per year. This may suggest a previously unknown association between neurofibromatosis type 1 and goblet cell carcinoids.

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