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Congenital fibrosarcoma is a rare fibrous tissue malignancy occurring most commonly in the extremities (71%), but presenting in axial locations as well (29%).
Congenital fibrosarcoma is unique among human sarcomas in that it has an excellent prognosis and very low metastatic rate.
It differs from the fibrosarcoma in adulthood by its clinical behaviour: the clinical course of congenital fibrosarcoma is more favorable and metastatic spread is rare. Localization also differs: while adult fibrosarcomas are common in the thigh, congenital fibrosarcomas affect chiefly the distal portions of the extremities.