Constantinoiu Silviu, Constantin Adrian, Predescu Dragos, Iosif Cristina, Hoara Petre, Achim Florin, Surugiu Paul, Bacanu Florin, Cociu Luminita
General and Esophageal Surgery Clinic, "Saint Mary's" Hospital Bucharest, Bucharest, Romania.
Hepatogastroenterology. 2012 Sep;59(118):1874-8. doi: 10.5754/hge11571.
Somatostatinoma is a rare neuroendocrine tumor which especially develops in the pancreas. There are few communicated cases about extra-pancreatic localization, having as a particularity the absence of somatostatin hypersecretion syndrome and frequent association with von Recklinghausen neurofibromatosis. We present the case of a 42-year old patient with Von Recklinghausen neurofibromatosis admitted in our clinic with a chronic upper digestive obstruction syndrome. The presence of a first jejunal loop somatostatinoma was an intraoperative surprising diagnosis that imposed jejunal resection and association of complementary specific treatment. Despite the therapeutic correct management, the status of the patient deteriorated very fast, confirming the aggressiveness of this neoplasia.
生长抑素瘤是一种罕见的神经内分泌肿瘤，尤其好发于胰腺。关于胰腺外定位的病例报道较少，其特点是不存在生长抑素分泌过多综合征，且常与冯·雷克林霍增氏神经纤维瘤病相关。我们报告一例42岁患有冯·雷克林霍增氏神经纤维瘤病的患者，因慢性上消化道梗阻综合征入住我院。术中意外诊断出首例空肠襻生长抑素瘤，遂行空肠切除术并联合辅助特异性治疗。尽管治疗管理正确，但患者病情仍迅速恶化，证实了这种肿瘤的侵袭性。
