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Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature.
Neurocirugia (Astur). 2009 Aug; 20(4):381-6; discussion 386-7.N

Abstract

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.

Authors+Show Affiliations

Division of Pediatric Neurosurgery, "12 de Octubre" Hospital, Madrid.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article
Review

Language

eng

PubMed ID

19688140

Citation

Otero-Rodríguez, A, et al. "Purely Intramedullary Spinal Cord Primitive Neuroectodermal Tumor: Case Report and Review of the Literature." Neurocirugia (Asturias, Spain), vol. 20, no. 4, 2009, pp. 381-6; discussion 386-7.
Otero-Rodríguez A, Hinojosa J, Esparza J, et al. Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature. Neurocirugia (Astur). 2009;20(4):381-6; discussion 386-7.
Otero-Rodríguez, A., Hinojosa, J., Esparza, J., Muñoz, M. J., Iglesias, S., Rodríguez-Gil, Y., & Ricoy, J. R. (2009). Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature. Neurocirugia (Asturias, Spain), 20(4), 381-6; discussion 386-7.
Otero-Rodríguez A, et al. Purely Intramedullary Spinal Cord Primitive Neuroectodermal Tumor: Case Report and Review of the Literature. Neurocirugia (Astur). 2009;20(4):381-6; discussion 386-7. PubMed PMID: 19688140.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Purely intramedullary spinal cord primitive neuroectodermal tumor: case report and review of the literature. AU - Otero-Rodríguez,A, AU - Hinojosa,J, AU - Esparza,J, AU - Muñoz,M J, AU - Iglesias,S, AU - Rodríguez-Gil,Y, AU - Ricoy,J R, PY - 2009/8/19/entrez PY - 2009/8/19/pubmed PY - 2009/11/17/medline SP - 381-6; discussion 386-7 JF - Neurocirugia (Asturias, Spain) JO - Neurocirugia (Astur) VL - 20 IS - 4 N2 - 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. SN - 1130-1473 UR - https://www.unboundmedicine.com/medline/citation/19688140/Purely_intramedullary_spinal_cord_primitive_neuroectodermal_tumor:_case_report_and_review_of_the_literature_ L2 - https://linkinghub.elsevier.com/retrieve/pii/7 DB - PRIME DP - Unbound Medicine ER -