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.

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