Dominguez-Comesaña Elias, Tome-Espiñeiro Catherine, Ulla-Rocha Jose L, Lorenzo-Lorenzo Isabel, Lede-Fernandez Angel, Portela-Serra Jose L
Surgery Service, Complejo Hospitalario de Pontevedra, Pontevedra, Spain.
Asia Pac J Clin Oncol. 2011 Sep;7(3):193-6. doi: 10.1111/j.1743-7563.2011.01419.x.
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.
壶腹类癌是一种罕见肿瘤，其中四分之一的病例在患有I型神经纤维瘤病的患者中被检测到。这种遗传性疾病还与胃肠道间质瘤（GIST）有关。然而，这三种实体共同出现的情况以前仅在五例中被检测到。一名53岁的女性患者，被诊断患有I型神经纤维瘤病，患有壶腹恶性类癌以及十二指肠和空肠的多个胃肠道间质瘤，接受了全胰切除术和肠道肿瘤切除术。五年后，随访显示患者情况良好，无肿瘤复发。壶腹类癌肿瘤、GIST和神经纤维瘤病同时存在非常罕见。根治性手术切除是一种很好的治疗选择，但对此的最佳管理尚未完全确立。
