目的：提高临床对超长型脊髓室管膜瘤的认识。方法报道经手术和病理证实的累及延髓和全脊髓的超长型室管膜瘤一例，观察其临床、影像及病理学特点及其治疗方式和预后。结果该病例颈椎和腰椎MRI显示第4脑室至腰2椎体水平脊髓内见弥漫性不均匀长T1、长T2信号影，脊髓内病灶明显强化，部分呈环形强化。计算机测量全部病变范围33 cm ×1.6 cm ×0.8 cm。全麻下行椎管探查术+脊髓内病变活检术+椎板减压术。术中病理诊断为脊髓室管膜瘤黏液乳头型（ WHO分级Ⅰ-Ⅱ级）。结论该病例是目前国内报道的病变范围最广的室管膜瘤，手术宜选取有明显强化囊变的部分切除。%Objective To investigate of clinical presentation,MR imaging and pathology features of super long intramedullary ependy-momas from the roof of fourth ventricular to the second vertebra. Methods A case of super long intramedullary ependymomas in fourth ventricular and spinal cord were reported. The clinical feature of super long intramedullary ependymomas was summarized and the related literature was re-viewed. Results MRI revealed a multinodular and multicystic lesion with enhancement in the medulla oblongala and nearly the whole spinal cord from the roof of the fourth ventricular to second vertebra. The lesion range was 33 cm × 1. 6 cm × 0. 8 cm. Intraoperative observation via cervical midline myelotomy revealed a grayish,gelatinous solid mass and part of the tumor revealed cystic formation. A pathological examination revealed the mass to be a ependymoma with mucinous papillary type( WHO gradeⅠ-Ⅱ). Conclusion This report presents the rare and the longest in-tramedullary ependymomas that has been reported in China,a review of the literature associated with super long intramedullary ependymomas,a discussion of the clinical and radiographical characteristics.
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