Congenital paraspinal Ewing sarcoma family of tumors with an epidural extension

Congenital Ewing sarcoma family of tumors (ESFT) is a rare disease, and only 12 patients have been reported. Among those patients, only two had paraspinal tumors with an epidural extension. A 3-week-old infant boy presented with a huge dorsal mass. Whole-spine MRI scans showed a paraspinal mass with an epidural extension from the T11 to L2 levels, causing severe spinal cord compression. An initial operation was performed to confirm the pathological diagnosis. Twenty days after the first operation, the patient showed left lower-extremity weakness. A second operation was performed with a laminectomy from the T11 to L2 levels, and the epidural mass was radically resected. Pathologically, the tumor was confirmed as an ESFT. The patient received adjuvant chemotherapy. His neurological deficit recovered after the second surgery, and there was no tumor recurrence during 17 months of follow-up.