Department of Neurosurgery, La Princesa University Hospital, Madrid, Spain.
Department of Neurosurgery, Puerta de Hierro University Hospital, Madrid, Spain.
Handb Clin Neurol. 2021;181:75-115. doi: 10.1016/B978-0-12-820683-6.00007-5.
The concept of craniopharyngiomas (CPs) primarily affecting the hypothalamus, or "hypothalamic CPs" (Hy-CPs), refers, in a restrictive sense, to the subgroup of CPs originally developing within the neural tissue of the infundibulum and tuber cinereum, the components of the third ventricle floor. This subgroup, also known as infundibulo-tuberal CPs, largely occupies the third ventricle and comprises up to 40% of this pathological entity. The small subgroup of strictly intraventricular CPs (5%), lesions wholly developed within the third ventricle above an anatomically intact third ventricle floor, can also be included within the Hy-CP category. The remaining types of sellar and/or suprasellar CPs may compress or invade the hypothalamic region during their growth but will not be considered in this review. Hy-CPs predominantly affect adults, causing a wide range of symptoms derived from hypothalamic dysfunction, such as adiposogenital dystrophy (Babinski-Fröhlich's syndrome), diabetes insipidus (DI), abnormal diurnal somnolence, and a complex set of cognitive (dementia-like, Korsakoff-like), emotional (rage, apathy, depression), and behavioral (autism-like, psychotic-like) disturbances. Accordingly, Hy-CPs represent a neurobiological model of psychiatric disorders caused by a lesion restricted to the hypothalamus. The vast majority (90%) of squamous-papillary CPs belong to the Hy-CP category. Pathologically, most Hy-CPs present extensive and strong adhesions to the surrounding hypothalamus, usually formed of a thick band of gliotic tissue encircling the central portion of the tumor ("ring-like" attachment) or its entire boundary ("circumferential" attachment). CPs with these severe adhesion types associate high surgical risk, with morbidity and mortality rates three times higher than those for sellar/suprasellar CPs. Consequently, radical surgical removal of Hy-CPs cannot be generally recommended. Rather, Hy-CPs should be accurately classified according to an individualized surgery-risk stratification scheme considering patient age, CP topography, presence of hypothalamic symptoms, tumor size, and, most importantly, the CP-hypothalamus adhesion pattern.
颅咽管瘤（CPs）主要影响下丘脑，或“下丘脑 CP”（Hy-CP），在狭义上是指最初在漏斗和灰结节的神经组织中发育的 CP 亚组，这是第三脑室底的组成部分。该亚组也称为漏斗-结节 CP，主要占据第三脑室，占该病理实体的 40%。严格位于第三脑室的小亚组（5%），即完全在解剖完整的第三脑室底上方的第三脑室中发育的病变，也可以归入 Hy-CP 类别。其余类型的鞍内和/或鞍上 CP 在生长过程中可能会压迫或侵犯下丘脑区域，但在本综述中不予考虑。Hy-CP 主要影响成年人，导致广泛的下丘脑功能障碍症状，如脂肪生殖营养不良（巴氏-弗罗利希综合征）、尿崩症（DI）、异常日间嗜睡以及一系列认知（痴呆样、柯萨科夫样）、情绪（愤怒、冷漠、抑郁）和行为（自闭症样、精神病样）障碍。因此，Hy-CP 代表了一种由局限于下丘脑的病变引起的精神障碍的神经生物学模型。绝大多数（90%）的鳞状乳头状 CP 属于 Hy-CP 类别。从病理上看，大多数 Hy-CP 与周围下丘脑有广泛而强烈的粘连，通常由环绕肿瘤中央部分的厚胶质带形成（“环状”附着）或其整个边界（“环形”附着）。具有这些严重粘连类型的 CP 与高手术风险相关，发病率和死亡率比鞍内/鞍上 CP 高 3 倍。因此，一般不建议对 Hy-CP 进行激进的手术切除。相反，应根据个体化的手术风险分层方案，根据患者年龄、CP topography、下丘脑症状存在情况、肿瘤大小，最重要的是 CP-下丘脑粘连模式，对 Hy-CP 进行准确分类。
