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成人起病的硬脊膜内脊髓畸胎瘤:单中心18例连续病例报告及预后分析

Adult-onset intradural spinal teratoma: report of 18 consecutive cases and outcomes in a single center.

作者信息

Wan Wei, Yang Cheng, Yan Wangjun, Liu Tielong, Yang Xinghai, Song Dianwen, Xiao Jianru

机构信息

Department of Bone Tumor Surgery, Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China.

Department of Orthopedics, Shanghai Jiao Tong University, Shanghai First People's Hospital, No.100 Haining Road, Shanghai, 200080, China.

出版信息

Eur Spine J. 2017 Jul;26(7):1917-1928. doi: 10.1007/s00586-016-4939-9. Epub 2017 Jan 21.

Abstract

STUDY DESIGN

Eighteen consecutive patients with adult-onset intradural spinal teratoma underwent surgical treatment in our center from 1998 to 2013.

BACKGROUND AND PURPOSE

Teratoma is defined as a neoplasm composed of elements derived from three germ cell layers (ectoderm, endoderm and mesoderm). Intraspinal teratoma is extremely rare and accounts for 0.2-0.5% of all spinal cord tumors. Moreover, teratoma occurs primarily in neonates and young children. Adult-onset intradural spinal teratoma is even rare. The aim of this study was to discuss the clinical characteristics, diagnosis and therapeutic strategies of adult-onset intradural spinal teratoma.

METHODS

This retrospective study included 18 consecutive adult patients with intradural teratoma who were surgically treated in our center between 1998 and 2013. The clinical features, pathogenesis, diagnostic strategies and surgical outcomes were discussed. Neurological function outcomes were evaluated by the JOA scoring system.

RESULTS

Of the 18 included patients, 4 patients received subtotal resection and the other 14 patients received total resection. All the 18 cases were diagnosed with mature teratoma. The mean follow-up period was 79.7 (median 60.5; range 27-208) months. Local recurrence occurred in two of the four patients who underwent subtotal resection and in no patient who underwent total resection. The neurologic status improved in 16 cases and remained unchanged in the other two patients.

CONCLUSIONS

Adult-onset intradural spinal teratoma is extremely rare. To the best of our knowledge, this is the largest series of patients with this disease. Despite the slow-growth and indolent nature, radical resection remains the recommended treatment to reduce tumor recurrence.

摘要

研究设计

1998年至2013年期间,我们中心对18例连续性成年发病的硬脊膜内脊髓畸胎瘤患者进行了手术治疗。

背景与目的

畸胎瘤被定义为由源自三个胚层(外胚层、内胚层和中胚层)的成分组成的肿瘤。脊髓内畸胎瘤极为罕见,占所有脊髓肿瘤的0.2 - 0.5%。此外,畸胎瘤主要发生于新生儿和幼儿。成年发病的硬脊膜内脊髓畸胎瘤更为罕见。本研究的目的是探讨成年发病的硬脊膜内脊髓畸胎瘤的临床特征、诊断及治疗策略。

方法

这项回顾性研究纳入了1998年至2013年期间在我们中心接受手术治疗的18例连续性成年硬脊膜内畸胎瘤患者。讨论了其临床特征、发病机制、诊断策略及手术结果。采用JOA评分系统评估神经功能结果。

结果

18例纳入患者中,4例行次全切除,另外14例行全切除。18例均诊断为成熟畸胎瘤。平均随访期为79.7(中位数60.5;范围27 - 208)个月。4例行次全切除的患者中有2例出现局部复发,而行全切除的患者无局部复发。16例患者神经状态改善,另外2例患者神经状态未变。

结论

成年发病的硬脊膜内脊髓畸胎瘤极为罕见。据我们所知,这是关于该疾病的最大病例系列。尽管其生长缓慢且惰性较强,但根治性切除仍是减少肿瘤复发的推荐治疗方法。

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