Treatment of cerebral Langerhans cell histiocytosis

Cerebral Langerhans cell histiocytosis (LCH) is a rare granulomatous disord er which may be primary or secondary or solitary or multiple. Brain structu res outside the hypothalamic-pituitary axis are only scarcely involved, eve n in multisystem varieties. Since there are neither controlled therapeutic trials nor systematic analyses of hitherto reported cases, optimal treatmen t strategies are not known. To evaluate the effect of different treatment m odalities, we analyzed previous reports of extrahypothalamic LCH back to 19 80 in which the diagnosis was made on the basis of examination of cerebral tissues. Thirty-five histologically examined cases were identified, includi ng 10 patients presenting with multiple cerebral lesions. Adding one own ca se followed up for 10 years, 16 patients had cerebral involvement secondary to multisystem LCH, while another 20 patients had primary cerebral LCH. Th e peak incidence was far beyond the pediatric range for both primary and se condary cerebral LCH. Localized lesions can be treated successfully by surg ery or radiation following biopsy. Chemotherapy may be an additional option . Multiple lesions can tentatively be controlled by chemotherapy and, possi bly, radiation. The ultimate outcome is determined by whether or not recurr encies or de-novo lesions will appear and the course of the systemic diseas e. Studies addressing the effects of therapy in cerebral LCH are urgently n eeded. (C) 1999 Elsevier Science B.V. All rights reserved.