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

被引:71
作者
Mobley, Bret C. [1 ]
Roulston, Diane
Shah, Gaurang V.
Bijwaard, Karen E.
McKeever, Paul E.
机构
[1] Stanford Univ, Med Ctr, Dept Pathol, Stanford, CA 94035 USA
[2] Univ Michigan, Med Ctr, Dept Pathol, Ann Arbor, MI 48901 USA
[3] Univ Michigan, Med Ctr, Dept Radiol, Ann Arbor, MI 48901 USA
[4] Armed Forces Inst Pathol, Dept Cellular Pathol, Washington, DC 20306 USA
关键词
peripheral primitive neuroectodermal tumor; Ewing's sarcoma; Cauda equina; MIC2; gene; EWS/FLI1gene; t(11; 22);
D O I
10.1016/j.humpath.2006.02.011
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
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 mail 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. (c) 2006 Elsevier Inc. All rights reserved.
引用
收藏
页码:845 / 853
页数:9
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