Coexistence of a Somatostatin-Producing Carcinoma of Duodenum and a Jejuna Gastrointestinal Stromal Tumor (GIST) in a Patient with Von Recklinghausen’s Disease

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Introduction: Duodenal neuroendocrine tumors comprise 2-3% of all neuroendocrine tumors of the gastrointestinal tract. Coexistence of NF-1, NET and GIST is rare and only five cases have been described in the literature.

Aim(s): To present a patient with the coexistence of Neurofibromatosis Type 1 (NF-1), somatostatinoma and stromal tumor of the gastrointestinal tract (GIST).

Materials and methods: A 55-year-old female patient presented with two tumors: one at the ampulla of Vater and a second at proximal jejunum. The presence of cafe-au-lait macules, neurofibromas, axillary freckling (Crowe sign), and Lisch nodules of the iris set the diagnosis of NF-1 in this patient. Therefore she was investigated for a neuroendocrine tumor.

Conference: 8th Annual ENETSConcerence (2011)

Presenting Author:

Authors: Daraki V, Stamou K, ?amalaki E, Spyrou F, Kalikakis G,

Keywords: somatostatinoma, Neurofibromatosis Type 1 , GIST, ,