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

被引：0

作者：

Otero-Rodriguez, A. ^{[1
]}

Hinojosa, J. ^{[1
]}

Esparza, J. ^{[1
]}

Munoz, M. J. ^{[1
]}

Iglesias, S. ^{[1
]}

Rodriguez-Gil, Y. ^{[1
]}

Ricoy, J. R. ^{[2
]}

机构：

[1] Hosp 12 Octubre, Div Pediat Neurosurg, E-28041 Madrid, Spain

[2] Hosp 12 Octubre, Dept Neuropathol, E-28041 Madrid, Spain

来源：

NEUROCIRUGIA | 2009年 / 20卷 / 04期

关键词：

Primitive neuroectodermal tumor; Spinal cord; Primary intramedullary; MEDULLOBLASTOMA; CHILDREN; BRAIN;

D O I：

暂无

中图分类号：

Q189 [神经科学];

学科分类号：

071006 ;

摘要：

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.

引用

页码：381 / 386

页数：6

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