" RECENT ADVANCES IN MANAGMENT OF EWING'S TUMOR-OF BONE "

Abstract

Ewing's tumor of bone was first described by James Ewing in 1921. Ewing's tumor of bone represents about 5% of all malignancies among children. It is the second most common primary malignant bone tumor in childhood after e>steosarcoma. ETB is high between ages 10 and 19, in males relative tq females, and is almost exclusively a disease of white children. Ewing's tumor of bone is characterized by the presence of chromosomal translocations. More than 85% of them harbor the translocation t(11 ;22) (q24; q 12), caused by fusion of EWS, FLI I genes. In 10% of cases, nother translocation is present; t(21;22) (q22;q12) caused by fusion of EWS, ERG genes. It shows also chromosomal amplifications in 75% of patients, including amplification of the long arm of chromosome 1 (1 q21- 1 q22) in 18% of patients, amplification of chromosome 8 in 38% of patients, and amplification of chromosome 12 in 12% or patients. Ewing's tumor of bone belongs to the Ewing's family of tumors (EFTs). In recent years, many investigators have favored a neuroectodermal origin for the tumor. The most common site of Ewing's tumor of bone is the femur, followed by the pelvis, followed by other long bones. Macroscopically, it is white, glistening, friable, and with large areas of hemorrhage and necrosis. On cut surface, there is gelatinous or mucoid material.