GRANULOCYTIC SARCOMA IN CHILDREN WITH ACUTE MYELOBLASTIC-LEUKEMIA ANDT(8-21)

Background. Granulocytic sarcomas (CS) have been associated with t(8;2 1). The prognosis of patients with CS is generally regarded as being l ess favorable than of patients with acute myeloblastic leukemia (AML). CS occurs relatively commonly in Africa and has been reported to affe ct 10-25% of black children presenting with AML. We sought to establis h the incidence of CS in our pediatric population, to determine whethe r an association with t(8;21) existed, and to report on the outcome of these cases in a single series. Procedure. The records of consecutive pediatric patients treated for de novo AML in Johannesburg between Ja nuary 1985-December 1995 were reviewed. Fifteen cases of CS among a to tal of 88 cases of AML presented to the Paediatric Haematology/ Oncolo gy Clinics of the Johannesburg and Baragwanath Hospitals. Fourteen (93 %) of these patients were black male children. Results. All 9 cases of orbital GS (60%) and almost all cases with concurrent AML M2 had t(8; 21). This translocation was present in only 4 (8.5%) of the remaining 47 AML cases without CS for which cytogenetic data were available. One case presented with a complex chromosomal translocation not previousl y associated with CS. The median disease-free survival of the CIS pati ents, using conventional chemotherapy treatment protocols, was signifi cantly better than for the patients with AML and no CS (P = 0.0004). C onclusions. Our data support a strong association between orbital CS, t(8;21), and AML M2 in the pediatric population. This entity occurred virtually exclusively in black male children at presentation. One thir d of these children who presented with AML had a CS. The favorable pro gnosis noted in our GS patients on standard induction and intensificat ion therapy without local irradiation conflicts with some previous rep orts but is consistent with the favorable outcome documented in AML wi th t(8;21). Med. Pediatr. Oncol. 31: 144-149, 1998. (C) 1998 Wiley-Lis s, Inc.