异位颅咽管瘤的诊断与治疗

Diagnosis and treatment of ectopic craniopharygioma

目的探讨异位颅咽管瘤(ectopic craniopharygioma)的临床诊断、手术疗效及预后,以期减少临床漏诊误诊。方法将收治的采用内镜经鼻入路手术治疗、病理确诊的3例异位颅咽管瘤患者的临床症状、影像学检查、手术方法以及预后情况进行总结。结果 3例患者发病部位分别位于蝶窦、颞下窝1例,随访22个月,分别采用2次内镜经鼻入路手术,并行放疗后肿瘤组织恶变致全身多器官功能衰竭死亡;后鼻孔、软腭1例,术后随访18个月,未见复发;颞下窝1例,术后随访3年,未见复发。结论异位颅咽管瘤非常罕见,诊断主要依靠病理及免疫组化检测。手术切除目前仍然作为首选治疗方式,是否应行放射治疗有待商榷。

Objective To investigate the diagnosis,surgical effect and prognosis of ectopic craniopharygioma so as to avoid misdiagnosis and missed diagnosis. Methods Clinical data( including clinical manifestations,imageological examination,surgical method and prognosis) of 3 patients with ectopic craniopharygioma surgically treated in our department from were analyzed retrospectively. Results The tumor was located in sphenoid sinus and infratemporal fossa in one patient and treated with two endonasal endoscopic surgeries and one auxiliary radiotherapy. The patient died of systemic multiple organ failure due to tumor malignant transformation in 22 months after treatment. The lesion location was posterior naris and soft palate in the second patient,and postoperative follow-up for 18 months revealed no recurrence. The lesion location was infratemporal fossa in the last case,and postoperative follow-up for 3 years showed no recurrence. Conclusion Ectopic craniopharygioma is very rare and its diagnosis mainly depends on pathology and immunohistochemistry. Surgical treatment should be the preferred method. Whether radiotherapy should be administered is still up for debate.