Congenital solitary fibromatosis of the skeleton.Case report of a variant of congenital generalized fibromatosis

A study of a 3-month-old boy with a solitary form of congenital generalized fibromatosis of the skeleton is presented. Radiographic examination revealed a shortening of the right ulna and an osteolytic lesion in the distal metaphysis extending to the epiphysis. The histological appearance seems to be consistent with previously described cases of generalized congenital fibromatosis of soft tissues and skeleton. Ultrastructurally, the tumor cells resembled primitive fibroblasts. The lesion was curetted 3 times and filled with bone transplants before it finally healed. At follow-up of the boy at age 13, there were no signs of the tumor.