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目的探讨主体位于后颅凹的颅咽管瘤的临床特点及手术治疗。方法回顾性分析9例后凹型颅咽管瘤的临床表现、影像学特点、手术治疗和随访结果。结果 9例肿瘤均为起源于鞍区而主体位于后颅凹的巨大型肿瘤,囊性2例,囊实性7例;肿瘤伸入桥小脑角8例,位于中线达下斜坡1例。头痛是最常见症状(6/9),颅神经损害较常见(4/9),内分泌功能障碍少见(3/9)。经幕上下联合入路全切除6例(7次手术),经扩大翼点入路全切和次全切各1例,枕下乙状窦后入路部分切除1例。无围术期死亡患者,8例患者恢复好。结论后凹型颅咽管瘤临床表现、影像学特点、手术入路、并发症与鞍区肿瘤有明显不同,可以考虑为分类中的一个新的亚型。
Objective To investigate the clinical features and surgical treatment of craniopharyngioma with the main body located in the posterior fossa. Methods The clinical manifestations, imaging features, surgical treatment and follow-up results of 9 cases of posterior craniopharyngioma were retrospectively analyzed. Results All the 9 tumors were giant tumors originating in the saddle area and located in the posterior fossa. There were 2 cystic cases and 7 cystic neoplasms. Tumors extended into the cerebellopontine angle in 8 cases and located in the midline down one slope. Headache is the most common symptom (6/9), cranial nerve damage is more common (4/9), and endocrine dysfunction is uncommon (3/9). 6 cases (7 surgeries) were removed by supratentorial approach, one by total excision and one by subtotal approach by means of enlarged pterional approach, and a partial resection by suboccipital sigmoid sinus approach. No perioperative deaths, 8 patients recovered well. Conclusions The clinical manifestations, imaging features, surgical approach, and complications of posterior craniopharyngioma are obviously different from those in the sellar region and can be considered as a new subtype in the classification.