Fibrosarcoma in infants and children: a retrospective analysis - overdiagnosis in earlier years

During a 30-year period, 22 patients considered to have a fibrosarcoma (FS) were treated. In a retrospective study the clinicopathologic findings were summarized. With histologic and immunohistochemical re-evaluation, the dia gnosis was confirmed in 8 cases. For 6 further patients FS was very probabl e but specimens were not available. In 8 cases the diagnosis was revised an d benign lesions were found in 7. Two patients with irresectable tumors die d (infantile FS, FS of mesentery and retroperitoneum). After repeated local recurrences and spread on the affected extremity, an amputation was life-s aving in 1 boy. In earlier years many tumors were classified as FSs. Today, immunohistochemistry and molecular-biological methods are valuable tools t o clearly identify these tumors. Wide local excision or en-bloc resection w ithout sacrificing any significant function of the part should be the prima ry form of treatment :in infants. Primary re-excision after incomplete exci sion should have priority over any adjuvant treatment. Preoperative chemoth erapy may avoid incomplete resection or mutilation in cases with extended c ongenital FS.