A CASE OF A DUODENAL SOMATOSTATINOMA ASSOCIATED WITH VON RECKLINGHAUSEN'S DISEASE

Abstract

Duodenal somatostatinoma is a relatively rare endocrine tumor and is associated with von Recklinghausen's disease. We report herein on the case of 36-year-old female who presented with upper gastrointestinal hemorrhage due to a duodenal somatostatinoma. She had café-au-lait spots and multiple subcutaneous neurofibromas. Endoscopic examination revealed a tumor with a hemorrhagic ulcer in the descending duodenum. The ulcer improved with conservative treatment, and a pylorus-preserving pancreaticoduodenectomy was performed. Pathological examination confirmed a carcinoid tumor with invasion of the pancreatic head and lymph node metastasis. Immunohistochemical analyses revealed that the tumor cells were positive for chromogranin A and somatostatin. The final diagnosis was a duodenal somatostatinoma associated with von Recklinghausen's disease.