Department of Endocrinology, Skånes University Hospital, 221 85, Lund, Sweden.
Pituitary. 2013 Mar;16(1):46-55. doi: 10.1007/s11102-012-0428-2.
A craniopharyngioma (CP) is an embryonic malformation of the sellar and parasellar region. The annual incidence is 0.5-2.0 cases/million/year and approximately 60 % of CP are seen in adulthood. Craniopharyngiomas have the highest mortality of all pituitary tumors. Typical initial manifestations at diagnosis in adults are visual disturbances, hypopituitarism and symptoms of elevated intracranial pressure. The long-term morbidity is substantial with hypopituitarism, increased cardiovascular risk, hypothalamic damage, visual and neurological deficits, reduced bone health, and reduction in quality of life and cognitive function. Therapy of choice is surgery, followed by cranial radiotherapy in about half of the patients. The standardised overall mortality rate varies 2.88-9.28 in cohort studies. Patients with CP have a 3-19 fold higher cardiovascular mortality in comparison to the general population. Women with CP have an even higher risk.
颅咽管瘤（CP）是鞍区和鞍旁区的胚胎性畸形。年发病率为 0.5-2.0 例/百万/年，约 60%的 CP 发生在成年期。颅咽管瘤是所有垂体瘤中死亡率最高的。成人诊断时的典型首发症状为视力障碍、垂体功能减退和颅内压升高的症状。长期发病率较高，包括垂体功能减退、心血管风险增加、下丘脑损伤、视力和神经功能缺损、骨健康状况下降，以及生活质量和认知功能降低。治疗方法首选手术，约一半患者随后行颅放射治疗。队列研究中标准化的总死亡率为 2.88-9.28。与普通人群相比，CP 患者的心血管死亡率高 3-19 倍。CP 女性患者的风险更高。
