Primary Intramedullary Ewing’s Sarcoma: A Case Report and Review of the Literature

Ewing’s sarcoma, a group of primary neuroectodermal tumor (PNET), is an uncommon rare highly malignant tumor mostly affecting the bones of male patients, and accounts for approximately 10% of the primary malignant bone tumors, and occupies the second place after osteosarcoma in children’s less than 20 years. Primary intramedullary Ewing’s sarcoma is an extremely rare condition. Here, we present a case of a 16-year-old girl with progressive weakness of lower extremities, inability to walk, progressive low back pain, bilateral leg pain, paresthesia and urinary retention. Spinal MRI showed intramedullary abnormal signal focus from T11 to L3 vertebrae with moderate heterogeneous enhancement on T1 weighted sequences. After surgery, histological examination found a lot of small round-cell tumors with a high nuclear-cytoplasmic ratio, frequent mitoses and apoptotic nuclei suggesting a diagnosis of Ewing’s sarcoma and confirmed by CD99 reactivity. She had undergone adjuvant chemotherapy and irradiation and was free of symptoms since 02 years. Within this single pure case of intramedullary Ewing’s sarcoma, perhaps the 1st in the English literature, the authors described this extremely rare uncommon localization with a brief review of the literature.