George H Sakorafas, George A Giannopoulos, Aikaterini Parasi, George Konstantoudakis, Nikolaos Tzanakis, Spiridon Stergiopoulos, George Peros
Context Somatostatin-producing endocrine tumors of the duodenum are very rare neoplasms of the gastrointestinal tract. These tumors may be associated with von Recklinghausen’s disease. Case report We present the case of a 49- year-old female patient with von Recklinghausen’s disease and an incidentally diagnosed ampullary neoplasm. The patient was treated with a classical pancreaticoduodenectomy. At surgery, a mass was found in the greater curve of the stomach which was resected using the classic Whipple procedure. Histology and immunohistochemistry showed that the duodenal tumor was an ampullary somatostatin-producing endocrine carcinoma while the gastric tumor was a gastrointestinal stromal tumor (GIST). The postoperative course was uneventful and the patient is alive, without tumor recurrence, six years after surgery. Conclusion Somatostatin-producing endocrine tumors of the duodenum are rare tumors, often associated with von Recklinghausen’s disease; these neoplasms should be treated aggressively using radical surgical resection. Although local resection may be appropriate for small duodenal somatostatin-producing tumors, a pancreaticoduodenectomy is usually required for larger tumors.
