Yamini Bakhtiar, Narayanan Malini
The University of Chicago Hospitals, Section of Neurosurgery, 5841 South Maryland Avenue, MC 4066, Chicago, IL 60637, USA.
Expert Rev Anticancer Ther. 2006 Sep;6 Suppl 9:S85-92. doi: 10.1586/14737140.6.9s.S85.
Craniopharyngiomas have been challenging practitioners for over 100 years. These benign cystic tumors are thought to be a part of a continuum of ectodermal cystic lesions found in the suprasellar region. They are rare tumors found in both the adult and pediatric populations. Patients most often present with findings associated with ventricular outflow obstruction causing hydrocephalus, optic chiasm compression resulting in visual dysfunction or hypothalamic/pituitary compression leading to endocrinopathy. The mainstay of treatment involves a combination of radical or limited surgery followed by radiotherapy. Other adjunctive strategies include cyst fenestration, Ommaya catheter implantation and brachytherapy. Patients in general have high long-term survival but can have significant tumor- and treatment-associated morbidity requiring a lifetime of medical and psychological management.
颅咽管瘤已经困扰从业者100多年了。这些良性囊性肿瘤被认为是鞍上区域发现的外胚层囊性病变连续体的一部分。它们是在成人和儿童群体中均有发现的罕见肿瘤。患者最常出现与脑室流出道梗阻导致脑积水相关的症状、视交叉受压导致视觉功能障碍或下丘脑/垂体受压导致内分泌病的症状。治疗的主要方法包括根治性或局限性手术联合放疗。其他辅助策略包括囊肿开窗术、奥马亚导管植入术和近距离放射治疗。总体而言,患者长期生存率较高,但可能有与肿瘤和治疗相关的严重发病率,需要终身的医疗和心理管理。