Case report: congenital retroperitoneal fibrosarcoma.

We report a case of congenital retroperitoneal fibrosarcoma presenting in a 17-day-old male in which surgical tumor debulking was followed by chemotherapy. His clinical course was complicated. Intraoperatively, 80% of the tumor was removed and he experienced massive blood loss and significant cardiac arrhythmias. Following surgery he received chemotherapy and suffered life-threatening hepatic, hematological and gastrointestinal toxicity. The treatment resulted in clinical remission after 9 months of follow-up. A review of the current literature revealed the changing opinion of the optimal treatment of fibrosarcomas in children. Chemotherapy is playing an increasingly important role in the treatment of fibrosarcoma, both preoperatively and postoperatively.