Isolated myelosarcoma of the skin in childhood

Background: isolated myelosarcoma of the skin in childhood is a rare manife station of acute myeloid leukemia (AML) preceding bone marrow involvement b y weeks to month. Results: During the studies of AML-BFM 87/93 for the treatment of acute mye loid leukemia an isolated myelosarcoma of the skin was diagnosed in eight p atients aged 0.3 to 16 years. Histologically most cases were classified as monoblastic or myelomonocytic leukemia (FAB M5, n=5; FAB M4, n=4; FAB MO, n =1). Two infants, 3 and 6 month old remained in complete remission after be ing treated only by surgical or by oral cytostatic therapy. Two children tr eated according to the AML-BFM-schedule are in complete remission. Delayed performance of diagnostic procedures and the initially mild symptoms seemed to be the reasons for reduced treatment in the remaining cases. After firs t relapse all patients received intensive cytostatic treatment (AML-BFM 87/ 93), but all of them relapsed a second time. Three of these children died d ue to progressive disease after 2 1/2 to 3 1/2 years. Only one patient is i n complete remission since 6 1/2 years. Conclusion: Suspect skin lesions should be considered as myelosarcoma prece ding AML. Reduced treatment seems to be justified only for young toddlers w ithout malignant bone marrow blasts. An early and intensive cytostatic trea tment is recommended for all other children with isolated myelosarcoma of t he skin.