Meyer Scott, Shah Shruti N, Dancel-Manning Kristen, Wang Yuxiu, Young Matthew, Agrawal Nidhi
Department of Medicine, NYU Langone Health, New York, NY, United States.
Department of Endocrinology, NYU Langone Health, New York, NY, United States.
Front Endocrinol (Lausanne). 2025 May 15;16:1527161. doi: 10.3389/fendo.2025.1527161. eCollection 2025.
Craniopharyngiomas are histologically benign central nervous system tumors derived from embryonic epithelial cells of Rathke's pouch. The disease demonstrates a bimodal age distribution, occurring most often in patients 5-14 and 50-74 years of age. Common comorbidities include hypopituitarism, hypothalamic obesity, sleep apnea, visual impairment and neurocognitive disturbances. There are several key differences in the presentation, tumor characteristics and clinical outcomes between age groups. Childhood craniopharyngiomas are mostly adamantinomatous and often present as larger tumors with worse functional outcomes such as rates of obesity and neurological deficits. Adults experience similar but slightly adjusted rates of comorbidity with both the adamantinomatous and papillary subtypes. This review presents a case-based discussion of adult craniopharyngiomas, focusing on recent literature regarding their presentation, pathology and pathogenesis, diagnosis, treatment and long-term sequelae.
颅咽管瘤是一种组织学上为良性的中枢神经系统肿瘤，起源于拉特克囊的胚胎上皮细胞。该疾病呈现双峰年龄分布，最常见于5至14岁以及50至74岁的患者。常见的合并症包括垂体功能减退、下丘脑性肥胖、睡眠呼吸暂停、视力障碍和神经认知障碍。不同年龄组在临床表现、肿瘤特征和临床结局方面存在一些关键差异。儿童颅咽管瘤大多为造釉细胞瘤，通常表现为较大的肿瘤，肥胖率和神经功能缺损等功能结局较差。成人患造釉细胞瘤和乳头型亚型的合并症发生率相似，但略有调整。本综述以病例为基础讨论成人颅咽管瘤，重点关注近期有关其临床表现、病理和发病机制、诊断、治疗及长期后遗症的文献。
