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黏液乳头型室管膜瘤手术切除后的长期预后。

Long term outcomes following surgical resection of myxopapillary ependymomas.

作者信息

Bagley Carlos A, Wilson Sean, Kothbauer Karl F, Bookland Markus J, Epstein Fred, Jallo George I

机构信息

Division of Neurosurgery, Duke University School of Medicine, Durham, NC, USA.

出版信息

Neurosurg Rev. 2009 Jul;32(3):321-34; discussion 334. doi: 10.1007/s10143-009-0190-8. Epub 2009 Feb 17.

Abstract

Myxopapillary ependymomas, a specific tumor variant of spinal cord ependymomas, occur most commonly in the lumbosacral region. During the study period, 1,013 patients underwent surgery for spinal cord tumors. Fifty-two of the patients had a myxopapillary ependymoma. Forty-eight of these patients underwent surgery at our institutions. There were four patients who came for consultations only. Fourteen pediatric patients were diagnosed with myxopapillary ependymoma. The overall average age at which a patient was diagnosed was 31.8 years. The average age a child was diagnosed was 12.6 years. The adult mean age was 38.7 years. The clinical presentation was of a slow, indolent course, with average symptom duration of 20.8 months. Overall, the pediatric patients had a much more aggressive clinical course with a much higher rate of local recurrence and dissemination of the tumor within the neural axis (64% versus 32%). The median time to disease recurrence was 88 months for the entire group. The overall survival after 11.5 years of follow-up was 94%. The optimal management of patients harboring myxopapillary ependymomas remains somewhat controversial. Excellent outcomes may be obtained, however, with the use of aggressive surgical techniques. No clear benefit for adjunctive chemotherapy, and radiation therapy was demonstrated.

摘要

黏液乳头型室管膜瘤是脊髓室管膜瘤的一种特殊肿瘤变体,最常发生于腰骶部。在研究期间,1013例患者接受了脊髓肿瘤手术。其中52例患者患有黏液乳头型室管膜瘤。这些患者中有48例在我们机构接受了手术。有4例患者仅前来咨询。14例儿科患者被诊断为黏液乳头型室管膜瘤。患者被诊断时的总体平均年龄为31.8岁。儿童被诊断时的平均年龄为12.6岁。成人平均年龄为38.7岁。临床表现为病程缓慢、进展隐匿,平均症状持续时间为20.8个月。总体而言,儿科患者的临床病程更具侵袭性,肿瘤在神经轴内的局部复发和播散率更高(64%对32%)。整个组疾病复发的中位时间为88个月。随访11.5年后的总体生存率为94%。对于患有黏液乳头型室管膜瘤的患者,最佳治疗方案仍存在一定争议。然而,采用积极的手术技术可能会取得良好的效果。辅助化疗和放射治疗未显示出明显益处。

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