L5 Corpectomy in a 12-Year-Old Owing to Rosai-Dorfman Disease: A Case Report

Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytosis typically presenting with massive, painless lymphadenopathy or subcutaneous masses. Extranodal involvement, particularly in the spine, is exceedingly rare and poses significant diagnostic and management challenges. Lumbar spinal RDD with vertebral collapse and neurological compromise is almost unheard of in the pediatric population, with no prior reports of surgical intervention requiring L5 corpectomy and circumferential fusion in an adolescent.

We present a case of a 12-year-old girl with a 6-month history of worsening lower back pain unresponsive to conservative treatment. Imaging revealed collapse of the L5 vertebral body and a destructive, enhancing lesion encasing the neural elements, leading to severe canal and foraminal stenosis. Computed tomography-guided biopsy confirmed the diagnosis of RDD. A multidisciplinary surgical team implemented a 2-stage circumferential surgical plan. Stage 1 involved posterior decompression and posterior instrumentation from L3 to the pelvis using S2AI screws. A silastic barrier was placed to protect neural elements. Stage 2, performed via an anterior approach with vascular surgery assistance, involved complete corpectomy of L5 and reconstruction with an expandable cage spanning L4 to S1. Postoperative recovery was uneventful, and the patient remained neurologically intact. At 1-year follow-up, the patient demonstrated solid anterior and posterior fusion, maintained spinal alignment, and had returned to full activity.

This is the first reported case of pediatric RDD requiring an L5 corpectomy with circumferential spinal fusion. The case underscores the importance of early tissue diagnosis, individualized surgical planning, and interdisciplinary collaboration. Recognition of RDD as a potential etiology for pediatric spinal lesions is critical, particularly when imaging mimics malignancy. Our successful outcome supports the use of staged, tailored surgical approaches in managing rare spinal pathologies in children.