Department of Pediatrics and Pediatric Hematology/Oncology, University Children´s Hospital, Carl von Ossietzky University Oldenburg, Klinikum Oldenburg AöR, 26133 Oldenburg, Germany.
J Clin Endocrinol Metab. 2021 Sep 27;106(10):e3820-e3836. doi: 10.1210/clinem/dgab397.
Craniopharyngiomas are rare embryonic malformational tumors of the sellar/parasellar region, classified by the World Health Organization (WHO) as tumors with low-grade malignancy (WHO I). The childhood adamantinomatous subtype of craniopharyngioma is usually cystic with calcified areas. At the time of diagnosis, hypothalamic/pituitary deficits, visual disturbances, and increased intracranial pressure are major symptoms. The treatment of choice in case of favorable tumor location (without hypothalamic involvement) is complete resection. It is important to ensure that optical and hypothalamic functionality are preserved. In case of unfavorable tumor location, that is with hypothalamic involvement, a hypothalamus-sparing surgical strategy with subsequent local irradiation of residual tumor is recommended. In the further course of the disease, recurrences and progression often occur. Nevertheless, overall survival rates are high at 92%. Severe impairment of quality of life and comorbidities such as metabolic syndrome, hypothalamic obesity, and neurological consequences can be observed in patients with disease- and/or treatment-related lesions of hypothalamic structures. Childhood-onset craniopharyngioma frequently manifests as a chronic disease so that patients require lifelong, continuous care by experienced multidisciplinary teams to manage clinical and quality of life consequences. For this review, a search for original articles and reviews published between 1986 and 2020 was performed in Pubmed, Science Citation Index Expanded, EMBASE, and Scopus. The search terms used were "craniopharyngioma, hypothalamus, pituitary obesity, irradiation, neurosurgery.
颅咽管瘤是鞍区/鞍旁区罕见的胚胎性发育畸形肿瘤，世界卫生组织（WHO）将其归类为低度恶性肿瘤（WHO I 级）。儿童型颅咽管瘤的造釉细胞瘤型通常为囊性伴钙化区。在诊断时，下丘脑/垂体功能减退、视力障碍和颅内压增高是主要症状。在有利的肿瘤位置（无下丘脑受累）的情况下，首选的治疗方法是完全切除。重要的是要确保保留视力和下丘脑功能。在不利的肿瘤位置（伴有下丘脑受累），建议采用下丘脑保护手术策略，随后对残留肿瘤进行局部放疗。在疾病的进一步发展过程中，常出现复发和进展。尽管如此，总体生存率仍高达 92%。由于下丘脑结构的疾病和/或治疗相关病变，患者可能会出现严重的生活质量受损和合并症，如代谢综合征、下丘脑性肥胖和神经后果。儿童期发病的颅咽管瘤常表现为慢性疾病，因此患者需要经验丰富的多学科团队提供终身、持续的护理，以管理临床和生活质量后果。为了进行本次综述，在 Pubmed、Science Citation Index Expanded、EMBASE 和 Scopus 中搜索了 1986 年至 2020 年期间发表的原始文章和综述。使用的搜索词是“颅咽管瘤、下丘脑、垂体性肥胖、放疗、神经外科”。
