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婴儿脊柱原发性非典型畸胎样/横纹肌样瘤

Primary atypical teratoid/rhabdoid tumor of the spine in an infant.

作者信息

Stabouli S, Sdougka M, Tsitspoulos P, Violaki A, Anagnostopoulos I, Tsonidis Ch, Koliouskas D

出版信息

Hippokratia. 2010 Oct;14(4):286-8.

Abstract

Atypical teratoid/rhabdoid tumor of the spine is a rare pediatric neoplasm with poor prognosis. We report a case of an atypical teratoid/rhabdoid tumor of the cervical spine in a 2-months-old infant. The patient presented with rapidly progressing tetraparesis and respiratory failure. Magnetic resonance imaging of the spinal cord revealed an intradural, extramedullary mass occupying the spinal canal on the right at the level of C1-C5. Tumor cells were immunohistochemically positive for epithelial membrane antigen, vimentin, cytokeratins, S-100 protein, and CD57/Leu-7 antigen. Despite chemotherapy, the infant presented with progressive neurological deterioration and died 6 months after initial diagnosis. We review the literature on spinal malignant rhabdoid tumor and discuss the pathology, treatment, and outcome of these rare neoplasms.

摘要

脊柱非典型畸胎样/横纹肌样瘤是一种预后不良的罕见儿童肿瘤。我们报告一例2个月大婴儿颈椎非典型畸胎样/横纹肌样瘤病例。该患者表现为快速进展的四肢瘫痪和呼吸衰竭。脊髓磁共振成像显示硬膜内、髓外肿块占据C1 - C5水平右侧椎管。肿瘤细胞上皮膜抗原、波形蛋白、细胞角蛋白、S - 100蛋白和CD57/Leu - 7抗原免疫组化呈阳性。尽管进行了化疗,该婴儿仍出现进行性神经功能恶化,并在初诊后6个月死亡。我们回顾了关于脊柱恶性横纹肌样瘤的文献,并讨论了这些罕见肿瘤的病理学、治疗方法及预后。

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