Our experience with childhood histiocytosis X

A retrospective analysis of the course of histiocytosis X in 16 children treated at our hospital over the past 15 years is presented. Nearly all of the patients were at generalized stages of the disease. In this study the staging according to Greenberg was used. The disease had a favorable course at Stages I and II, 1 patient at Stage III and 2 at Stage IV died. All the children were treated by chemotherapy, in some of them combined with radiotherapy. Individual patients differed from one another with respect to chemotherapeutic regimens. The actuarial survival rate was 78% for the whole group. Better results were obtained after a less intense but long-term treatment than after an intense but interrupted chemotherapy.