Surgical Management of Metastatic Extraskeletal Extradural Ewing Sarcoma of the Thoracic Spine in a Patient with Wiskott-Aldrich Syndrome and Hypertrophic Cardiomyopathy: A Case Report.

Background Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disorder increasing the risk of malignancy, commonly lymphoma. Extraskeletal Ewing sarcoma (EES) is rare, typically occurring in the trunk, extremities, and retroperitoneum. Features of EES include rapid growth, quick metastasis, and localized pain. Observations A 31-year-old male with a history of WAS and hypertrophic cardiomyopathy (HCM) reported to the emergency department for a 1-day onset of difficulty ambulating, bilateral lower extremity weakness, numbness ascending to the thorax, and mid-back pain. A T5 sensory level was established with genitalia sensation and bulbocavernosus reflexes absent. Magnetic resonance imaging (MRI) with and without contrast of the cervical, thoracic, and lumbar spine revealed a contrast-enhancing mass with extraosseous extension producing severe spinal stenosis and cord compression. Acute neurological deterioration necessitated emergency decompression. Intraoperative pathology showed a small blue-cell tumor concerning for neuroectodermal tumor. Total resection was attempted with T1-T3 fusion and right T2 transpedicular costotransversectomy. Posttumor debulking, ambulation and sensation were regained. Pathological specimens were consistent with EES. Lessons WAS increases the risk for lymphoma, though broad differentials should be considered, as neurosurgical debulking may be required for neurological recovery in non-radiosensitive tumors. HCM and WAS increase intraoperative complexity and the need for interdisciplinary collaboration in oncological therapy.