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[一例无病生存期极长的腰骶部室管膜瘤转移:病例报告及文献复习]

[Metastasis of a lumbosacral ependymoma with very long disease-free survival: a case report and review of the literature].

作者信息

Castadot Pierre, Magné Nicolas, Roelandts Martine, Gastelblum Pauline, Oulad Ben Taib Nordeyn, Van Houtte Paul

机构信息

Département de radiothérapie-oncologie, institut Jules-Bordet, 121, boulevard de Waterloo, 1000 Bruxelles, Belgique.

出版信息

Cancer Radiother. 2006 May;10(3):148-51. doi: 10.1016/j.canrad.2006.02.003. Epub 2006 Apr 3.

Abstract

Ependymoma is rare glial tumour of the central nervous system and is considered to be low-grade. The lumbosacral location of spinal ependymoma is the most common. Prognosis of ependymomas is dependent on tumour location, histological subtype and differentiation, extent of the tumour and of the completeness of the surgical resection. One of the characteristics of this kind of tumour is to present the possibility of a seeding of the entire cerebrospinal axis, by the way of cerebrospinal liquid. We describe the case of a young male patient operated by incomplete resection of a lumbar ependymoma. Six months later, the patient's symptoms reappeared and an external radiotherapy at curative doses and chemotherapy were delivered. Evolution of the remaining tumour was diagnosed 6 years after at the primary site and operated by large incomplete resection. A second session of radiotherapy was therefore administered. Twenty-four years after this episode, cervical pain and gait troubles appear. Complete imaging study concluded to a cervical extramedullary intradural tumour and to the persistence of the primary lumbosacral tumour. Macroscopical complete resection of the cervical tumour was performed and pathological findings concluded to a metastasis of his lumbar ependymoma. External radiotherapy was delivered on this site with a total dose of 50 Gy. Eight years after this episode, the patient is alive without evidence of distant disease. The primary lumbosacral ependymoma is stable. Ependymomas are often recurrent at the primary site, but can seed on the entire cerebrospinal axis. Awareness of such aberrant tumoral behaviour, even after such a long disease free interval, may warrant more careful follow-up of patients with this diagnosis.

摘要

室管膜瘤是中枢神经系统罕见的神经胶质瘤,被认为是低级别肿瘤。脊髓室管膜瘤最常见于腰骶部。室管膜瘤的预后取决于肿瘤位置、组织学亚型和分化程度、肿瘤范围以及手术切除的完整性。这类肿瘤的一个特点是有可能通过脑脊液在整个脑脊髓轴上播散。我们描述了一例年轻男性患者,其腰椎室管膜瘤手术切除不完全。6个月后,患者症状复发,接受了根治性剂量的外照射放疗和化疗。6年后在原发部位诊断出残留肿瘤进展,并进行了大范围不完全切除手术。因此进行了第二轮放疗。这次事件24年后,患者出现颈部疼痛和步态障碍。完整的影像学检查显示为颈椎髓外硬膜内肿瘤,且原发腰骶部肿瘤仍然存在。对颈椎肿瘤进行了肉眼下完全切除,病理结果显示为腰椎室管膜瘤转移。对该部位进行了外照射放疗,总剂量为50 Gy。这次事件8年后,患者存活,无远处疾病证据。原发腰骶部室管膜瘤病情稳定。室管膜瘤常在原发部位复发,但也可在整个脑脊髓轴上播散。即使在如此长的无病间隔期后,认识到这种异常的肿瘤行为可能需要对诊断为此病的患者进行更仔细的随访。

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