Pediatric Spinal Epidural Lymphoma Presenting with Compressive Myelopathy: A Distinct Pattern of Disease Presentation

Objective: Spinal epidural lymphoma with compressive myelopathy is a rarely found condition. The aims of this study are to describe the clinical features and to analyze its treatment outcome and prognostic factors.

Methods: We searched for all pediatric patients with newly diagnosed spinal epidural lymphoma from 1999 to 2014 in our institution. We evaluated the clinical features, including neurologic status, time interval to treatment, treatment modality, and outcomes.

Results: Twelve of 302 pediatric patients with lymphoma (4.0%) presented with compressive myelopathy, and they were all found to have spinal epidural lymphoma. In 11 patients, epidural space was the only site of lymphoma involvement. The median age was 9 years (range, 5-15 years). Common initial symptoms were back pain and low extremity weakness. Surgery was performed on 9 patients, biopsy on 2 patients, and radiation therapy on 1 patient. In 9 patients who received surgery, 6 patients with preoperative motor power grade ≥II attained improvement in weakness. Three patients with preoperative motor power grade <II had no improvement in weakness at 1 month postoperatively. One patient who received radiation therapy had no improvement. All patients who had surgery developed some degree of kyphotic deformity later.

Conclusions: Spinal epidural lymphoma with compressive myelopathy is a serious disease in children necessitating urgent treatment. Preserved motor power is a meaningful prognostic factor of neurologic improvement. Treatment should be individualized based on the patient's neurologic condition.