Abstract
Peripheral primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (ES) of the central nervous system is extremely rare and should be differentiated from central PNET and other small blue round cell tumors. We describe a case of a meningeal peripheral PNET/ES of the spinal cord in an 11-year-old boy. Immunohistochemically, the small blue round cell tumor showed expression of epithelial markers and of CD99, thus posing an important differential diagnostic problem with a poorly differentiated synovial sarcoma. Fluorescence in situ hybridization revealed rearrangement of the EWS gene, as seen in peripheral PNET/ES. Peripheral PNET/ES does occur in the central nervous system, but its diagnosis can be extremely difficult on morphologic and immunohistochemical grounds alone. Genetic analysis plays a key role in its distinction from other small blue round cell tumors.
MeSH terms
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Antineoplastic Combined Chemotherapy Protocols / administration & dosage
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Biomarkers, Tumor / metabolism
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Bone Neoplasms / genetics
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Bone Neoplasms / metabolism
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Bone Neoplasms / pathology*
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Bone Neoplasms / therapy
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Child
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Diagnosis, Differential
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Humans
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Keratins / metabolism*
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Laminectomy
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Male
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Meninges / pathology*
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Mucin-1 / metabolism
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Neuroectodermal Tumors, Primitive / genetics
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Neuroectodermal Tumors, Primitive / metabolism
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Neuroectodermal Tumors, Primitive / pathology*
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Neuroectodermal Tumors, Primitive / therapy
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Sarcoma, Ewing / genetics
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Sarcoma, Ewing / metabolism
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Sarcoma, Ewing / pathology*
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Sarcoma, Ewing / therapy
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Sarcoma, Synovial / diagnosis
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Spectral Karyotyping
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Spinal Cord / surgery
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Spinal Cord Neoplasms / genetics
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Spinal Cord Neoplasms / metabolism
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Spinal Cord Neoplasms / pathology*
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Spinal Cord Neoplasms / therapy
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Vimentin / metabolism
Substances
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Biomarkers, Tumor
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Mucin-1
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Vimentin
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Keratins