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原发性脊柱硬膜外骨外尤文肉瘤

Primary spinal epidural extraosseous Ewing's sarcoma.

作者信息

Kaspers G J, Kamphorst W, van de Graaff M, van Alphen H A, Veerman A J

机构信息

Department of Pediatrics, Free University Hospital, Amsterdam, The Netherlands.

出版信息

Cancer. 1991 Aug 1;68(3):648-54. doi: 10.1002/1097-0142(19910801)68:3<648::aid-cncr2820680335>3.0.co;2-q.

DOI:10.1002/1097-0142(19910801)68:3<648::aid-cncr2820680335>3.0.co;2-q
PMID:2065287
Abstract

The case of a 7-year-old boy with a spinal epidural extraosseous Ewing's sarcoma (EES) is presented. He is in complete remission without neurologic deficit 40 months after diagnosis. Another 15 cases were found in the literature and are discussed together with this patient. Twelve of them were male patients. The mean age of the patients was 17.5 years (range, 4 to 47). Symptoms included back pain and/or radicular pain (100%), paresis of one or both legs (83%), sensory disturbances, and bladder and bowel dysfunction. The mean diagnostic delay was 5.8 months. Each patient underwent laminectomy; complete resection of the tumor was impossible in more than 50% of the cases. Most patients received radiation therapy and/or chemotherapy. Four patients suffered from local recurrence, eight from metastases. Ten (63%) patients died, 1 to 48 months (mean, 16) after diagnosis. The differential diagnosis is discussed, including disk herniation and several benign and malignant tumors.

摘要

本文报告了一例7岁男孩患有脊柱硬膜外骨外尤文肉瘤(EES)的病例。诊断后40个月,他处于完全缓解状态,无神经功能缺损。在文献中还发现了另外15例病例,并与该患者一起进行讨论。其中12例为男性患者。患者的平均年龄为17.5岁(范围4至47岁)。症状包括背痛和/或神经根性疼痛(100%)、单腿或双腿无力(83%)、感觉障碍以及膀胱和肠道功能障碍。平均诊断延迟为5.8个月。每位患者均接受了椎板切除术;超过50%的病例无法完全切除肿瘤。大多数患者接受了放射治疗和/或化疗。4例患者出现局部复发,8例出现转移。10例(63%)患者在诊断后1至48个月(平均16个月)死亡。文中讨论了鉴别诊断,包括椎间盘突出以及几种良性和恶性肿瘤。

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Primary spinal epidural extraosseous Ewing's sarcoma.原发性脊柱硬膜外骨外尤文肉瘤
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