Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature

Otero-Rodríguez A.; Hinojosa J.; Esparza J.; Mu?oz M.J.; Iglesias S.; Rodríguez-Gil Y.; Ricoy J.R.

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Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature

机译：单纯髓内脊髓原始神经外胚层肿瘤：病例报告及文献复习

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Introduction. Primitive neuroectodermal tumors (PNETs) are malign neoplasms of the central nervous system which mainly locate in cerebellum (medulloblastoma). Primary intraspinal PNETs are rare. Within this group, we have found ten cases of purely intramedullary PNETs (IPNETs). In this report, we describe a new IPNET case and review the literature about these infrequent intramedullary tumors. Case report. A 17 month-old boy showed progressive decrease of motion in his lower extremities. Spine magnetic resonance imaging revealed an intramedullary expansive lesion from T3 to T10. A near-total removal was performed. The pathological diagnosis was PNET. Subsequent chemotherapy was recommended. Six months after operation, holocord progression has occurred. Conclusion. IPNETs are uncommon tumors affecting children and young adults. They are characterized by recurrence, progression or intracranial dissemination. Outcome is dismal: most patients die within two years in spite of surgical resection followed by radiotherapy and chemotherapy.

机译：介绍。原始神经外胚层肿瘤（PNET）是中枢神经系统的恶性肿瘤，主要位于小脑（髓母细胞瘤）中。原发性脊柱内PNET很少见。在该组中，我们发现了十例纯髓内PNET（IPNET）。在本报告中，我们描述了一个新的IPNET病例，并回顾了有关这些罕见髓内肿瘤的文献。案例报告。一个17个月大的男孩下肢运动逐渐减少。脊柱磁共振成像显示从T3到T10的髓内扩张病变。几乎全部去除。病理诊断为PNET。建议随后进行化疗。手术六个月后，出现了全膝关节疾病进展。结论。 IPNET是影响儿童和年轻人的罕见肿瘤。它们的特点是复发，进展或颅内扩散。结果令人沮丧：尽管进行了外科手术切除，然后放疗和化疗，但大多数患者在两年内死亡。

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《Neurocirugia》 |2009年第4期|共7页
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Otero-Rodríguez A.; Hinojosa J.; Esparza J.; Mu?oz M.J.; Iglesias S.; Rodríguez-Gil Y.; Ricoy J.R.;
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Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature

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