Purohit Devendra, Chanduka Amit Kumar, Sharma Vinod, Mittal Radhey Shyam, Singhvi Shashi
Departments of Neurosurgery, and Pathology, SMS Medical College, Jaipur, Rajasthan, India.
Asian J Neurosurg. 2014 Oct-Dec;9(4):239. doi: 10.4103/1793-5482.146640.
Juvenile Xanthogranuloma (JXG) is a rare disorder of central nervous system. It rarely produces compressive myelopathy. On reviewing world literature, we could find only nine cases of this disease involving spine and of which only four cases were in adults' i.e., 18 years and above. We are presenting a case of Spinal JXG in an 18-year-old male with thoracic compressive myelopathy presenting as short duration progressive paraparesis. Magnetic Resonance Imaging of Spine showed mass lesion in epidural space compressing cord from behind without any bony involvement at D7 to D10 vertebral segment. It was isointense on T1 and hyperintense on T2 with no contrast enhancement. D7 to D10 Laminectomy with complete excision of firm epidural mass was carried out. The histopathology with tumor markers confirmed the diagnosis of JXG. Post-operative neurological recovery in this patient was good. His power improved to grade 5/5 with decreased spasticity. Follow-up MRI at 3 months showed no residual tumor. This case appears to be the first in the series with entirely extradural component in adult thoracic spine.
青少年黄色肉芽肿(JXG)是一种罕见的中枢神经系统疾病。它很少引起压迫性脊髓病。查阅世界文献时,我们仅发现9例该疾病累及脊柱,其中仅4例为成人,即18岁及以上。我们报告一例18岁男性的脊柱JXG病例,其表现为短期进行性截瘫的胸段压迫性脊髓病。脊柱磁共振成像显示硬膜外间隙有肿块病变,从后方压迫脊髓,在D7至D10椎体节段无任何骨质受累。它在T1加权像上呈等信号,在T2加权像上呈高信号,无强化。实施了D7至D10椎板切除术并完整切除硬膜外坚实肿块。肿瘤标志物的组织病理学检查确诊为JXG。该患者术后神经功能恢复良好。其肌力改善至5/5级,痉挛减轻。术后3个月的随访磁共振成像显示无残留肿瘤。该病例似乎是成人胸椎完全硬膜外成分系列中的首例。