title: Coincidence of GIST and pancreatic endocrine neoplasm in neurofibromatosis.
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.
authors: Surgery Service, Complejo Hospitalario de Pontevedra, Pontevedra, Spain. eliasdomcom@gmail.com
authors: Elias
authors: E
authors: Dominguez-Comesaña
authors: Catherine
authors: C
authors: Tome-Espiñeiro
authors: Jose L
authors: JL
authors: Ulla-Rocha
authors: Isabel
authors: I
authors: Lorenzo-Lorenzo
authors: Angel
authors: A
authors: Lede-Fernandez
authors: Jose L
authors: JL
authors: Portela-Serra
mesh_terms: D014670:Ampulla of Vater / Q000473:pathology* / Q000601:surgery; D005260:Female; D046152:Gastrointestinal Stromal Tumors / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008875:Middle Aged; D017253:Neurofibromatoses / Q000473:pathology* / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000473:pathology* / Q000601:surgery