Primary intracranial Ewing sarcoma with an unusually aggressive course: a case report and review of the literature.
The occurrence of Ewing sarcoma-peripheral primitive neuroectodermal tumor as a primary intracranial tumor is very rare, with only 29 cases reported in the literature, 19 of which have included molecular studies. We present the clinical, radiologic and pathologic findings of an intracranial Ewing sarcoma in a 22-year-old woman arising from the dura over the right frontal convexity. The patient underwent craniotomy with gross total excision of the tumor. The tumor showed atypical histology and the diagnosis was confirmed by detection of a rearrangement of the EWSR1 gene by fluorescent in situ hybridization and identification of the diagnostic t(11;22)(q24;q12) translocation by reverse transcription-polymerase chain reaction. Additional features were detected in this tumor that are known to be associated with an unfavorable prognosis, including loss of p16 expression and gains of chromosomes 1q and 12. The patient experienced the most rapid downhill course reported to date for intracranial Ewing sarcoma, developing multiple extracranial metastases at 2 months and dying 6 months after the initial operation.
Department of Pathology, Siriraj Hospital, Bangkok, Thailand. jtanboon@gmail.com
Jantima
J
Tanboon
Bunpot
B
Sitthinamsuwan
Tewajetsada
T
Paruang
Paula
P
Marrano
Paul S
PS
Thorner
D000912:Antibodies, Neoplasm / Q000032:analysis / Q000378:metabolism; D001932:Brain Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D002148:Calmodulin-Binding Proteins / Q000235:genetics; D003399:Craniotomy; D018450:Disease Progression; D004388:Dura Mater / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008279:Magnetic Resonance Imaging; D009336:Necrosis; D009362:Neoplasm Metastasis / Q000473:pathology; D009363:Neoplasm Proteins / Q000378:metabolism; D016133:Polymerase Chain Reaction; D011379:Prognosis; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics; D012512:Sarcoma, Ewing / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D014057:Tomography, X-Ray Computed; D051598:Whole Body Imaging; D055815:Young Adult
D000912:Antibodies, Neoplasm; D002148:Calmodulin-Binding Proteins; C500986:EWSR1 protein, human; D009363:Neoplasm Proteins; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins