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Coincidence of GIST and pancreatic endocrine neoplasm in neurofibromatosis
Author(s) -
DOMINGUEZCOMESAÑA Elias,
TOMEESPIÑEIRO Catherine,
ULLAROCHA Jose L,
LORENZOLORENZO Isabel,
LEDEFERNANDEZ Angel,
PORTELASERRA Jose L
Publication year - 2011
Publication title -
asia‐pacific journal of clinical oncology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 29
eISSN - 1743-7563
pISSN - 1743-7555
DOI - 10.1111/j.1743-7563.2011.01419.x
Subject(s) - ampulla of vater , gist , neurofibromatosis , medicine , duodenum , stromal tumor , multiple endocrine neoplasia , pancreatectomy , ampulla , pancreas , neurofibromin 1 , gastroenterology , stromal cell , radiology , carcinoma , biology , biochemistry , gene
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.