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J Pediatr Surg. 1987 Jul;22(7):665-70.

Congenital fibrosarcoma.


Congenital fibrosarcoma is a rare fibrous tissue malignancy occurring most commonly in the extremities (71%), but presenting in axial locations as well (29%). Two of our five patients had lower extremity tumors and three had axial lesions located in the thoracolumbar region and anterior neck. Amputation was considered necessary to treat the extremity tumors. Two of the patients with axial lesions had incomplete resection, which raised the question of adjunctive therapy and prompted this review. Forty-seven additional cases of fibrosarcoma, present at birth or diagnosed within the first 3 months of life, were adequately described and documented in the literature giving a total of 52 cases. There was no sex predominance (male 26, female 20, unknown 6). Thirty-seven (71%) of the patients had extremity lesions. The local recurrence rate for extremity tumors was 32% with metastatic and mortality rates of 8% and 5%, respectively. Axial tumors (15 cases, 29%) had a local recurrence rate of 33%, which is similar to that for extremity lesions, but the metatstatic and mortality rates were 26% each indicating a more aggressive behavior for axial lesions. The overall mortality rate of 11.5% and metastatic rate of 13.5% for congenital fibrosarcoma, are not dissimilar to the figures quoted for older children. This pattern of tumor behavior, therefore, does not seem to warrant a different attitude because of the "congenital" occurrence of the tumor. Surgery must be considered the mainstay of therapy for fibrosarcoma, but there is a need for adjunctive therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

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