HISTIOCYTOSIS-X - CLINICAL ASPECTS AND SU RGICAL THERAPY

Histiocytosis X is a disease that is predominantly seen in infants and small children. As it is rarely manifested in older children and adul ts, the clinician may have difficulty in establishing the differential diagnosis. The clinical presentation can be unilocular or multilocula r and limited to the skeletal system or a disseminated form with organ involvement. The symptoms are variable and often uncharacteristic. Hi stology is necessary for a definite diagnosis. To determine the approp riate therapy and prognosis of histiocytosis X the extent of the disea se needs to be verified by additional diagnostic examinations. Eosinop hilic granulomas can normally be treated with local surgical intervent ion, whereas with the disseminated form chemotherapy is preferred. Fou r cases studies from our institution will be presented including diagn osis confirmation, staging examinations and therapy.