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.

A 3‐year‐old male with an extramedullary, intra‐ and extradural mass at T11‐L1

“…Six of the spinal JXG were in an extramedullary, intradural localization, six in vertebral bodies, and one in the cauda equina. These findings in pediatric patients are not different from those in 13 reported adult patients with spinal JXG: no definite predilection of sex or location of the lesion is observed [2]. The average age of the adult group is 30.3 years with an age range of 18–67 years.…”