Extramedullary myeloid tumors in XXXX : the limited value of local treatment.

@entity1 @entity5 @entity4586 @entity1600 @entity693 @entity828 @entity3052
PURPOSE: To determine the incidence of @entity5 ( @entity5 ) in Saudi Arabian @entity1 with @entity3052 , the factors associated with these @entity5 and the impact of local treatment on @entity5 control, complete remission and survival rates. @entity1 AND METHODS: One hundred @entity1 , median age 6 years, who received their primary treatment for @entity3052 at King Faisal Specialist Hospital and Research Center, from 1983 to 1997 were studied. @entity5 at diagnosis occurred in 18 (18%) @entity1 at 25 sites. @entity693 , @entity828 , @entity1600 , @entity4586 , and cutaneous infiltration were not included in the definition of @entity5 . With these exclusions, @entity1 with @entity5 were younger than those without @entity5 (median age, 3.5 v. 7.5 years) and were more likely to have @entity693 at diagnosis (33% v. 10%). The t(8;21) translocation was associated with a 47% @entity5 incidence compared with 23% without the translocation. Local radiation treatment was given to 16 of 25 (64%) @entity5 sites. RESULTS: The overall 5-year survival rate for all @entity1 was 28%, and this was not significantly influenced by the drug regimen used, @entity693 at diagnosis, the presence of the (8;21) translocation, M4 and M5 morphology combined, or @entity5 at diagnosis. Significant differences were observed in the 5-year survival rates for @entity1 who underwent allogeneic bone marrow transplantation (52%; N = 37) and those who attained complete remission (CR) but did not undergo transplantation (21%; N = 44) and those who did not achieve complete remission with initial therapy (5%; N = 19). Systemic and local @entity5 CR was achieved in 17 of 18 @entity1 with @entity5 , including 12 @entity1 who underwent radiation treatment and 5 of 6 of those who did not. Isolated relapse was not seen at an @entity5 site and was not noted at any later stage of the disease. CONCLUSIONS: Permanent local control at sites of @entity5 was achieved in all @entity1 who attained a bone marrow CR, whether or not the site was irradiated. Local radiation treatment of an @entity5 site did not appear to contribute to overall CR and survival rates. The use of radiation treatment should be conservative and limited to @entity1 in whom there is a real and immediate threat to vision or renal function or when the spinal cord is compromised.
