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Vol. 20. Issue 4.
Pages 381-386 (January 2009)
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Vol. 20. Issue 4.
Pages 381-386 (January 2009)
Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature
Tumor neuroectodérmico primitivo espinal exclusivamente intramedular: caso clínico y revisión de la literatura
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A. Otero-Rodríguez
, J. Hinojosa, J. Esparza, M.J. Muñoz, S. Iglesias, Y. Rodríguez-Gil
Division of Pediatric Neurosurgery. “12 de Octubre” Hospital. Madrid
J.R. Ricoy*
* Department of Neuropathology. “12 de Octubre” Hospital. Madrid
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Summary
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.

Keywords:
Primitive neuroectodermal tumor
Spinal cord
Primary intramedullary
Abreviaturas:
CNS
GFAP
IPNET
MCNU
MRI
NSE
PNET
VP-16
WHO
Resumen
Introducción

Los tumores neuroectodérmicos primitivos (PNETs) son neoplasias malignas del sistema nervioso central que principalmente se localizan a nivel del cerebelo (denominadas en este caso meduloblastomas). Son muy poco frecuentes los PNETs primarios intraespinales. Dentro de este grupo, solamente hemos encontrado diez casos de PNETs exclusivamente intramedulares. En este artículo, describimos un nuevo caso de IPNET y procedemos a revisar la literatura existente sobre este tipo de tumores.

Caso clínico

Se trata de un niño de 17 meses de edad que ha desarrollado una paraparesia progresiva. La resonancia magnética espinal muestra una lesión expansiva intramedular que se extiende desde T3 hasta T10. Se procedió a una extirpación macroscópica casi completa. El estudio anatomopatológico reveló un PNET. Se recomendó quimioterapia. Seis meses después de la cirugía, ha habido progresión a lo largo de la médula.

Conclusión

Los PNETs exclusivamente intramedulares son raras neoplasias que afectan a niños y adultos jóvenes. Se caracterizan por la recurrencia, progresión o diseminación intracraneal. Son procesos de muy mal pronóstico, ya que los pacientes mueren en los primeros dos años a pesar de la resección quirúrgica y posterior radioterapia y quimioterapia.

Palabras clave:
Tumor neuroectodérmico primitivo
Médula espinal
Primario intramedular

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