Peripheral primitive neuroectodermal tumor/Ewing's sarcoma of the craniospinal vault: case reports and review.

Mobley BC, Roulston D, Shah GV, Bijwaard KE, McKeever PE
Hum Pathol. 2006 37 (7): 845-53

PMID: 16784984 · DOI:10.1016/j.humpath.2006.02.011

The peripheral primitive neuroectodermal tumor/Ewing's sarcoma family tumor (pPNET/ESFT) group includes small round cell tumors of the bone, soft tissue, and nerve with morphological attributes of the germinal neuroepithelium. Peripheral PNETs/ESFTs also occur within the craniospinal vault, a region including the central nervous system, the meninges, and the cranial and spinal nerve roots. Gene rearrangements between the EWS gene on chromosome 22q12 and members of the ETS gene family are common in and specific to pPNETs/ESFTs. Another defining characteristic of pPNETs/ESFTs is their membranous expression of the MIC2 gene product. We describe 2 cases of pPNETs within the craniospinal vault. An intradural tumor arising from the nerve roots of the cauda equina was discovered in a 32-year-old man presenting with radiculopathic back pain and lower-extremity weakness. An intracranial pPNET that mimicked a meningioma was found in a 21-year-old man presenting with headache and visual disturbances. MIC2 gene product expression and EWS/ETS gene rearrangement were detected in both case patients. The literature with regard to pPNETs/ESFTs arising within the craniospinal vault is reviewed.

MeSH Terms (22)

12E7 Antigen Adult Antigens, CD Back Pain Blotting, Southern Brain Neoplasms Cauda Equina Cell Adhesion Molecules Chromosome Aberrations Chromosomes, Human, Pair 22 Diagnosis, Differential Headache Humans Male Meningioma Neuroectodermal Tumors, Primitive, Peripheral Oncogene Proteins, Fusion Peripheral Nervous System Neoplasms Proto-Oncogene Protein c-fli-1 Reverse Transcriptase Polymerase Chain Reaction RNA-Binding Protein EWS Sarcoma, Ewing

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