Non-Functioning Somatostatinoma of the Duodenum in Patient with Von Recklinghausen's Disease

ABSTRACT

Somatostatinoma is a rare endocrine tumor that comprises around 1% of all gastroenteropancreatic endocrine neoplasm. The estimated annual incidence is 1 in 40 million. This tumor may be associated with von Recklinghausen's disease. We present here a rare case of a 51-year-old female patient with a duodenal nonfunctioning somatostatinoma combined with von Recklinghausen's disease. Whipple' procedure was performed. The postoperative course was uneventful and the histopathologic findings were consistent with malignant nonfunctioning somatostatinoma with lymph node metastases. The patient is alive, healthy and without tumor recurrence 10 months after surgery.