Treatment of cerebral Langerhans cell histiocytosis

Cerebral Langerhans cell histiocytosis (LCH) is a rare granulomatous disorder which may be primary or secondary or solitary or multiple. Brain structures outside the hypothalamic-pituitary axis are only scarcely involved, even in multisystem varieties. Since there are neither controlled therapeutic trials nor systematic analyses of hitherto reported cases, optimal treatment strategies are not known. To evaluate the effect of different treatment modalities, we analyzed previous reports of extrahypothalamic LCH back to 1980 in which the diagnosis was made on the basis of examination of cerebral tissues. Thirty-five histologically examined cases were identified, including 10 patients presenting with multiple cerebral lesions. Adding one own case followed up for 10 years, 16 patients had cerebral involvement secondary to multisystem LCH, while another 20 patients had primary cerebral LCH. The peak incidence was far beyond the pediatric range for both primary and secondary cerebral LCH. Localized lesions can be treated successfully by surgery or radiation following biopsy. Chemotherapy may be an additional option. Multiple lesions can tentatively be controlled by chemotherapy and, possibly, radiation. The ultimate outcome is determined by whether or not recurrencies or de-novo lesions will appear and the course of the systemic disease. Studies addressing the effects of therapy in cerebral LCH are urgently needed.