Wisnu Baskoro, - and Muhammad Fakhri Raiyan Pratama, - and Ayu Yoniko Christi, - and Muhammad Faris, Muhammad and Eko Agus Subagio, Eko and Pandu Wicaksono, - and Bidari Kameswari, - Case of Compressive Myelopathy due to Juvenile Xanthogranuloma of Cervicothoracic Junction in a 28-year-old-male. Surgical Neurology International, 14 (17). pp. 1-6. ISSN 2152-7806

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Abstract

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.

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