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  • Title: Coincidence of GIST and pancreatic endocrine neoplasm in neurofibromatosis.
    Author: Dominguez-Comesaña E, Tome-Espiñeiro C, Ulla-Rocha JL, Lorenzo-Lorenzo I, Lede-Fernandez A, Portela-Serra JL.
    Journal: Asia Pac J Clin Oncol; 2011 Sep; 7(3):193-6. PubMed ID: 21884431.
    Abstract:
    Carcinoids of the ampulla of Vater are infrequent tumors of which a quarter of cases have been detected in patients with type I neurofibromatosis. This hereditary disease is also associated with gastrointestinal stromal tumors (GIST). However, the coincidence of these three entities together have only been formerly detected in five cases. A 53 year-old female patient, diagnosed with type I neurofibromatosis, with a malignant carcinoid of ampulla of Vater and multiple gastrointestinal stromal tumors in the duodenum and jejunum, was treated with total pancreatectomy and the excision of her intestinal tumors. Five-years on, a follow-up showed the patient to be well, and free from tumor recurrence. The coexistence of an ampullary carcinoid tumor, GIST and neurofibramatosis is very rare. Radical curative surgical resection is a good treatment option, but the optimal management of this is not yet well established.
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