LANGERHANS CELL HISTIOCYTOSIS

Langerhans cell histiocytosis is a non-neoplastic granulomalike lesion of unknown origin; it is characterized by proliferation of reticulohi stiocytic structures, polynuclear eosinophils, neutrophils, lymphocyte s, plasma cells, multinucleate giant cells and Langerhans cells. In re cent years, the older terms ''eosinophilic granuloma'' and ''histiocyt osis X'' have been replaced by the more precise expression ''Langerhan s cell histiocytosis''. The granuloma can be solitary and multifocal, with or without soft-tissue involvement. A specific entity is Hand-Sch uller-Christian disease. Characteristic lesions in the skull are combi ned with exophthalmus and diabetes insipidus. These symptoms occur bec ause of the involvement of the hypophysis. A clinically malignant form of the disease is Letterer-Siwe dis ease. This syndrome occurs in ver y small children. Dysfunction of various organs causes early death of the individual. The etiology of Langerhans cell histiocytosis is proba bly a dysfunction of the immune system. In the 130 cases registered at the Swiss bone tumor reference center the lesions were localized in a lmost all bones. The mandibula, skull, ribs and femur were the most fr equently involved bones. Eighty-three patients (64%) were male, 43 (36 %) female. Seventy-five percent of the lesions occurred in the first three decades of life. Ninety-three lesions were monostotic and 24 pol yostotic; in 5 cases there was soft-tissue involvement, 7 had Hand-Sch iiller-Christian, and 1 Letterer-Siwe disease. Treatment of osseous le sions should be purely surgical; there is no need for chemotherapy in such cases. If soft-tissue involvement is diagnosed, chemotherapy shou ld be considered. Alternatively, cortisone or interferone therapy can be administered. In our opinion there is no place for radiation therap y.