Coincidence of GIST and pancreatic endocrine neoplasm in neurofibromatosis
| Autor: | Jose Luis Ulla-Rocha, Isabel Lorenzo-Lorenzo, José Luis Portela-Serra, Catherine Tome-Espiñeiro, Ángel Lede-Fernandez, Elias Dominguez-Comesaña |
|---|---|
| Rok vydání: | 2011 |
| Předmět: |
medicine.medical_specialty
Stromal cell GiST business.industry Ampulla of Vater General Medicine Pancreatic Endocrine Neoplasm medicine.disease digestive system Gastroenterology digestive system diseases Jejunum medicine.anatomical_structure Oncology Internal medicine medicine Duodenum In patient Neurofibromatosis business neoplasms |
| Zdroj: | Asia-Pacific Journal of Clinical Oncology. 7:193-196 |
| ISSN: | 1743-7555 |
| DOI: | 10.1111/j.1743-7563.2011.01419.x |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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