Central Nervous System Histiocytosis X — Imaging and Responses to Chemotherapy

Abstract

Histiocytosis X is the term first coined by Lichtenstein in 19531 to describe a heterogeneous group of disorders which is considered now to include Hand-Schuller-Christian disease, Letterer-Siwe disease and Eosinophilic Granuloma of bone. Gagel, in 1941, first described involvement of the central nervous system (CNS) in Histiocytosis X2 - in this case the hypothalamus and posterior pituitary were the areas principally affected. CNS involvement outwith these areas is rare, generally difficult to diagnose3, and little information on treatment is available. In this case we describe a man with cranial histiocytosis X who was treated with intrathecal and systemic chemotherapy and cranial irradiation, and we comment upon the value of magnetic resonance imaging (MRI) in this condition.