Duodenal somatostatinoma and gastrointestinal stromal tumor associated with neurofibromatosis

Susan Kruse Sullivan, RN, Alan L. Schuricht, MD, Jill D. Siegfried, MS, Jean M. Fede, DO, and Teresa Victoria, MD

Departments of Surgery, Oncology, Pathology, and Radiology, The Joan Karnell Cancer Center, Pennsylvania Hospital, Philadelphia, PA

Somatostatinomas, a rare form of a malignant somatostatin-producing neuroendocrine tumor, are found with increasing frequency in patients with neurofibromatosis type 1 (NF1). Like somatostatinomas, gastrointestinal stromal tumors (GISTs) seem to occur with higher frequency in patients with NF1. However, a causative link between NF1 and both types of gastrointestinal tumors has not been elucidated. In most cases, somatostatinoma is a pathologic diagnosis made after surgery. The prognosis for patients with duodenal somatostatinoms, which are typically the less aggressive variety, is more favorable than the prognosis for those with other duodenal and pancreatic malignancies. Much of the literature suggests that treatment for these types of tumors generally involves an aggressive surgical approach, even in the presence of nodal or localized metastasis.

Commun Oncol 2006;3:266–270 print e-mail full text 175 kb