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  • Title: Large somatostatin-producing endocrine carcinoma of the ampulla of vater in association with GIST in a patient with von Recklinghausen's disease. Case report and review of the literature.
    Author: Sakorafas GH, Giannopoulos GA, Parasi A, Konstantoudakis G, Tzanakis N, Stergiopoulos S, Peros G.
    Journal: JOP; 2008 Sep 02; 9(5):633-9. PubMed ID: 18762695.
    Abstract:
    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.
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