Korean J Hepatobiliary Pancreat Surg.  2009 Dec;13(4):307-310.

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

• Kim KJ
• Jang KT
• Kim WS
• Choi DW

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.

Keyword

Neuroendocrine tumors; Somatostatinoma; Von Recklinghausen's disease

MeSH Terms

Duodenum
Female
Humans
Incidence
Lymph Nodes
Middle Aged
Neoplasm Metastasis
Neuroendocrine Tumors
Neurofibromatosis 1
Recurrence
Somatostatinoma