Isolated intracranial juvenile xanthogranuloma. A report of two cases and review of the literature

Excerpt

Juvenile xanthogranuloma (JXG) is a benign cutaneous disorder of non-Langerhans cell histiocytic proliferation which usually appears in children with a predilection for the head and neck region and is generally self-limiting. The first reports in the literature of JXG are credited to Adamson in 1905 and McDonagh in 1912 [1]. While the majority of cases occur in the first 6 months of life, up to 20 % occur in adolescents and young adults [2]. Although in over 90 % of cases, patients with JXG present as a solitary cutaneous lesion, sometimes it may involve internal organs such as the liver, spleen, lungs, kidneys, eyes, subcutaneous soft tissue, bones, and central nervous system (CNS), with or without cutaneous lesions associated, and may have an aggressive clinical course [3]. …