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


METHODS
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.


CONCLUSIONS
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.
Otero-Rodríguez
A
A
Division of Pediatric Neurosurgery, "12 de Octubre" Hospital, Madrid.
Hinojosa
J
J
Esparza
J
J
Muñoz
M J
MJ
Iglesias
S
S
Rodríguez-Gil
Y
Y
Ricoy
J R
JR
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D016190:Carboplatin / Q000008:administration & dosage; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D018450:Disease Progression; D005047:Etoposide / Q000008:administration & dosage; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage; D018242:Neuroectodermal Tumors, Primitive / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D020335:Paraparesis / Q000209:etiology; D011379:Prognosis; D013120:Spinal Cord Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D013904:Thoracic Vertebrae
D005047:Etoposide; D003520:Cyclophosphamide; D016190:Carboplatin; D002945:Cisplatin; D008727:Methotrexate