Intracerebral Langerhans cell histiocytosis treated with low dose Radiation alone: report of a rare case and review of the literature

Cerebral Langerhans cell histiocytosis (LCH) is a rare granulomatous disorder which may be primary, secondary and solitary or multiple. Central Nervous System (CNS) involvement without a systemic disease is very rare. The most frequent involvement of the cerebral LCH is in the cranial bones and the hypothalamic-pituitary axis (HPA). Cerebral LCH without involvement of HPA is very rare. We have brought into focus the biology and treatment options of this rare tumor by citing a case of intracerebral LCH (involving periventricular, parenchymal, midbrain, external capsule, and putamen) without involving HPA in a 10-year-old boy. He was treated successfully with radiotherapy. A short review of the relevant literature is also presented. We have also outlined the biology and the therapeutic options of this enigmatic tumor.