Solitary Intracranial Juvenile Xanthogranuloma Masquerading as Low-Grade Glioma - A Rare Case Report.

Juvenile xanthogranuloma (JXG) is a rare, benign histiocytic disorder of young children. It is most often present with cutaneous involvement of the head, neck and trunk region. Systemic JXG causes cutaneous lesions with extracutaneous involvements frequently seen in the orbit, liver, spleen, lung, kidney and bones. Central nervous system (CNS) involvement is uncommon and is usually seen as a component of systemic disease. Isolated JXG of CNS is very rare and only few cases have been reported till date. Here we report a case of isolated solitary intracranial JXG mimicking clinically and radiologically as a low-grade glioma with no signs of cutaneous or other systemic involvement. Gross total excision of the tumour was done and final diagnosis was made by histopathological and immunohistochemical examination. CNS involvement of JXG can cause significant morbidity and mortality. These cases are usually misdiagnosed on radiology as glioma or meningioma and the diagnosis is usually made by histopathology. Surgery is the mainstay of treatment and these patients should be on long-term follow-up since the natural history of the disease is still unknown. The case is presented here for its rarity and for its clinical significance.