Case of compressive myelopathy due to juvenile xanthogranuloma of cervicothoracic junction in a 28-year-old male

Abstract

Background: Juvenile xanthogranuloma (JXG) is a proliferative disorder of non-Langerhans histiocytes. The lesions typically occur in children as solitary cutaneous lesions, but are only rarely found in adults in their late twenties to thirties. Approximately 5–10% of JXG are extracutaneous in location, with spinal JXG being only rarely encountered. Here, we described a 28-year-old male with an extradural spinal JXG resulting in severe C6– T1 spinal cord compression and a progressive quadriparesis that warranted a decompressive laminectomy/C6–T2 fusion. Case Description: A 28-year-old male presented with a progressive quadriparesis of 12 months’ duration that rapidly worsened over the last 3 months. When the MRI revealed severe cord epidural C6–T1 cord compression, the patient successfully underwent a C6–T1 laminectomy for gross total tumor excision followed by a C6–T2 instrumented fusion. The histopathology confirmed the diagnosis of a spinal JXG. Conclusion: Spinal JXGs in adults are only rarely encountered and should be treated with gross total tumor excision with/without fusion to achieve the best long-term outcomes.