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J Pediatr Neurosci. 2019 Jan-Mar;14(1):46-51. doi: 10.4103/jpn.JPN_72_18.

Isolated Langerhans Cell Histiocytosis Masquerading as Intradural Extramedullary Meningioma: Review on Histiocytic Disorders of Spine.

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Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, Uttar Pradesh, India.
MDM Hospital and Medical College, Jodhpur, Rajasthan, India.
Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, Uttar Pradesh, India.


The histiocytic disorders are pathological diagnosis and rarely affects spine. The spinal involvement is characterized by lytic lesions and painful symptoms. Isolated intradural extramedullary involvement is rare presentation. A 15-year-old female patient presented with nontraumatic cervical compressive myelopathy and was operated electively with preoperative diagnosis of meningioma. The histopathology was surprisingly Langerhans cell histiocytosis. In lineage of histiocytic development, the Langerhans cells develop into matured dendritic cells and lose its Birbeck granules and CD1a antigenicity. With the understanding of histiocyte lineage system, the disorders concerned with central nervous system are classified into dendritic cell disorders and macrophages-related disorders. In this article, we have discussed on histiocytic disorders of central nervous system and management guidelines in case one comes across such histopathology.


Dendritic cell disorders; Erdheim–Chester disease; Langerhans cell histiocytosis; Rosai–Dorfman disease; hemophagocytic lymphohistiocytosis; juvenile xanthogranuloma

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