Landriel Federico, Ajler Pablo, Tedesco Nicolas, Bendersky Damián, Vecchi Eduardo
Neurosurgical Department of the Hospital Italiano de Buenos Aires. J.D. Perón, CH - Buenos Aires - Argentina.
Surg Neurol Int. 2012;3:102. doi: 10.4103/2152-7806.100859. Epub 2012 Sep 13.
Ependymoma has been described typically as an intramedullary tumor derived from ependymal cells. Intradural extramedullary presentation is rarely described and almost always as a unique lesion. Myxopapillary ependymoma is a histological variant that distinguishes from the ordinary type of ependymoma because of its generally better prognosis. We present two cases of multicentric extramedullary myxopapillary ependymomas.
Case 1 was a 30-year-old man with progressive paresthesia and paresis in the lower limbs, urinary sphincter disturbances, gait instability, ataxia, and chronic low back pain with multiple intradural extramedullary lesions at C2-C3, D2-D4-D5, and D12-L1. Case 2 was a 32-year-old man, presented with low back pain and mild paresthesia in the right lower limb. Magnetic resonance imaging (MRI) showed multiple intradural extramedullary lesions with homogeneous enhancement after gadolinium injection at C7, D2, D4, D5, D8, D10, D11, L1, L3, L5, S1, and S2. Complete tumor resection of the approached tumors was archived in both cases. Histological studies confirm myxopapillary ependymomas. Patient's neurologic outcome was good and no residual tumor was present at MRI control at 10 years in case 1 and 12 months in case 2.
We report the first two cases of multicentric extramedullary myxopapillary ependymomas, this etiology must be taken into account in the differential diagnosis of intradural extramedullary tumors.
室管膜瘤通常被描述为起源于室管膜细胞的髓内肿瘤。硬膜内髓外表现很少被描述,几乎总是作为一种独特的病变。黏液乳头型室管膜瘤是一种组织学变异型,因其预后通常较好而与普通型室管膜瘤相区别。我们报告两例多中心性髓外黏液乳头型室管膜瘤病例。
病例1为一名30岁男性,出现下肢进行性感觉异常和无力、尿失禁、步态不稳、共济失调以及慢性下背痛,在C2 - C3、D2 - D4 - D5和D12 - L1处有多个硬膜内髓外病变。病例2为一名32岁男性,表现为下背痛和右下肢轻度感觉异常。磁共振成像(MRI)显示在C7、D2、D4、D5、D8、D10、D11、L1、L3、L5、S1和S2处有多个硬膜内髓外病变,注射钆后呈均匀强化。两例均对所累及的肿瘤进行了完整切除。组织学研究证实为黏液乳头型室管膜瘤。病例1在10年的MRI复查时患者神经功能预后良好且无残留肿瘤,病例2在12个月时情况相同。
我们报告了首例两例多中心性髓外黏液乳头型室管膜瘤病例,在硬膜内髓外肿瘤的鉴别诊断中必须考虑到这种病因。