Primary spinal epidural undifferentiated round cell sarcoma with Ewing-like features in an 8-year-old child: Diagnostic challenges and recovery after subtotal resection.
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BACKGROUND: Primary epidural extraosseous Ewing sarcoma (EES) is exceptionally rare in children. Some tumors display the morphological and immunophenotypic features of EES but lack the canonical Ewing sarcoma breakpoint region 1 (EWSR1) rearrangement, creating a diagnostic dilemma. CASE DESCRIPTION: An 8-year-old girl presented with worsening nocturnal lumbosacral pain, paraparesis (3/5), and urinary incontinence. Magnetic resonance imaging revealed a large dumbbell-shaped epidural mass extending from L3 to S1 with homogeneous contrast enhancement. Emergency L2-S1 laminotomy allowed piecemeal subtotal resection because of intimate circumferential adherence to the dura mater and emerging nerve roots. Histopathology demonstrated small round blue cells with strong CD99 positivity, whereas markers for lymphoma, neuroblastoma, and rhabdomyosarcoma were negative. Fluorescence in situ hybridization for EWSR1 rearrangement was negative, and the tumor was classified as an undifferentiated round cell sarcoma with Ewing-like features. The patient received Vincristine, Adriamycin, Cyclophosphamide Ifosfamide, Etoposide (VAC/IE) chemotherapy according to the European Ewing tumour Working Initiative of National Groups (EUROE.W.I.N.G.) protocol and achieved full neurological recovery. CONCLUSION: This case illustrates the diagnostic and therapeutic challenges posed by Ewing-like sarcomas in the absence of molecular confirmation. Function-preserving subtotal resection combined with multimodal chemotherapy may lead to excellent outcomes and may be preferable to radical resection when the latter carries a high neurological risk.