Chapter 7 - Craniopharyngiomas primarily affecting the hypothalamus

Abstract

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.