title: Meningeal Ewing Sarcoma/Peripheral PNET: Clinicopathological, Immunohistochemical and FISH study of four cases.
abstract: Meningeal Ewing Sarcoma (ES)/peripheral primitive neuroectodermal tumor (pPNET) is a rare diagnostically challenging small round cell tumor in the CNS. This study investigates the clinical pathological features of four cases of this tumor from archives of 6 years in our hospital. Patients were within the median age of 21.5 years and male to female ratio was 1:1. The tumors distributed at the supra-tentorial location, posterior fossa and lumbar vertebral canal, usually presenting as the dura-sited nodule or having close connection with the meninges within the cranium or vertebral canal. Histopathologically, small round undifferentiated tumor cells with hypercellularities, scant cytoplasm and inconspicuous nucleoli were observed, although some components such as atypical larger vesicular nuclei, prominent nucleoli of tumor cells, necrotic foci and mesenchymal collagen proliferation forming the lobular structure, were also appreciated. Immunohistochemally, tumor cells displayed membranous positivity of CD99 (4/4), nuclear positivity of FLI-1 (4/4) and NKX2.2 (4/4), negativity of EMA, GFAP and synaptophysin expression. The histochemical PAS staining showed weak positivity in one case. Fluorescence in situ hybridization (FISH) test using EWSR1 (22q12) dual color break apart rearrangement probe showed positive results in two cases. Results suggest that using a panel of immunohistochemical markers, including NKX2.2, CD99, FLI-1, EMA, GFAP and synaptophysin, combined with the supplementary EWSR1 FISH test, helps to define the diagnosis of meningeal ES/pPNET of CNS.
authors: Department of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
authors: Changshu
authors: Ch
authors: Ke
authors: Department of Biochemistry and Molecular Biology, School of Basic Medicine, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
authors: Qiuhong
authors: Q
authors: Duan
authors: Department of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
authors: Hui
authors: H
authors: Yang
authors: Department of Biochemistry and Molecular Biology, School of Basic Medicine, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
authors: Feng
authors: F
authors: Zhu
authors: Department of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
authors: Meng
authors: M
authors: Yan
authors: Department of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
authors: San-Peng
authors: SP
authors: Xu
authors: Department of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
authors: Sheng
authors: S
authors: Zhou
authors: Department of Neurosurgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
authors: Feng
authors: F
authors: Wan
authors: Department of Neurosurgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
authors: Kai
authors: K
authors: Shu
authors: Department of Neurosurgery, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
authors: Ting
authors: T
authors: Lei
authors: Department of Radiology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China.
authors: Li-Ming
authors: LM
authors: Xia
mesh_terms: D000071443:12E7 Antigen / Q000378:metabolism; D000293:Adolescent; D000328:Adult; D014408:Biomarkers, Tumor / Q000235:genetics / Q000378:metabolism; D002148:Calmodulin-Binding Proteins / Q000235:genetics / Q000378:metabolism; D004388:Dura Mater / Q000378:metabolism / Q000473:pathology; D005260:Female; D000090322:Homeobox Protein Nkx-2.2; D018398:Homeodomain Proteins / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D008297:Male; D008577:Meningeal Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D009687:Nuclear Proteins; D051789:Proto-Oncogene Protein c-fli-1 / Q000378:metabolism; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics / Q000378:metabolism; D012512:Sarcoma, Ewing / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D014157:Transcription Factors / Q000378:metabolism; D029961:Zebrafish Proteins
chemical_list: D000071443:12E7 Antigen; D014408:Biomarkers, Tumor; D002148:Calmodulin-Binding Proteins; C500986:EWSR1 protein, human; C078433:FLI1 protein, human; D000090322:Homeobox Protein Nkx-2.2; D018398:Homeodomain Proteins; C000715631:NKX2-2 protein, human; D009687:Nuclear Proteins; D051789:Proto-Oncogene Protein c-fli-1; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins; D014157:Transcription Factors; D029961:Zebrafish Proteins; C000715630:nkx2.2b protein, zebrafish
keywords: Ewing sarcoma; FISH; immunohistochemistry; meningeal; pathology