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 reportA 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.
ConclusionIPNETs 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.
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 clínicoSe 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.
ConclusiónLos 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.
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