MALIGNANT HISTIOCYTIC DISORDERS IN CHILDREN - CLINICAL AND THERAPEUTIC APPROACHES WITH A NOSOLOGIC DISCUSSION

Malignant histiocytic disorders, other than leukemias, are extremely r are in childhood. Despite unresolved nosologic and terminologic diffic ulties, they should be classified according to the Lineage of the aber rant cells in a given tumor. There are no common and typical clinical presentations, nor are there established treatment modalities availabl e. For the disseminated forms, aggressive systemic treatment modalitie s-similar if not identical to those used for large cell anaplastic lym phomas-appear to be the best treatment option. For the localized forms , which are primarily dendritic cell sarcomas, a more localized and in dividualized therapy is appropriate.