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Congenital solitary fibromatosis of the skeleton. Case report of a variant of congenital generalized fibromatosis
Author(s) -
Kindblom LarsGunnar,
Angervall Lennart
Publication year - 1978
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(197802)41:2<636::aid-cncr2820410229>3.0.co;2-6
Subject(s) - medicine , fibromatosis , metaphysis , skeleton (computer programming) , lesion , ulna , anatomy , epiphysis , axial skeleton , pathology
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.