Abstract
Congenital fibrosarcoma (CFS) is a rare tumor most often affecting extremities of babies. Considering age, surgery of primary is preferred. Nevertheless amputation rate remains high. Preoperative chemotherapy (CT) role must be emphasised. We present 3 cases receiving preoperative CT.
Patients and methods in 1985, we treated a 3 months old girl for CFS of the thigh. To avoid amputation, preoperative CT (3 Ifosfamide- Vincristine- Actinomycine D) was performed leading to complete radiological and histological response. She benefited of conservative surgery She is in first complete remission 23 years later.
In September 1999, a 3 ½ y old boy with recurrent l buttock CFS operated elsewhere twice (6 months old, 2 years old), received preoperative chemotherapy with good clinical and radiological response. “En-bloc” extra tumoral resection was performed. Histology showed viable tumoral cells. We completed treatment by chemotherapy. In 01/ 2003 bilateral pulmonary metastases occurred leading to surgery and chemotherapy. In 09/ 2003 a new local recurrence appeared treated by surgery and postoperative chemotherapy. From this time, he received Alpha interferon. He is in complete remission for 6 years.
In 12/2005, a 14 y old girl, with local recurrence of CFS, treated elsewhere at the age of 5 months by partial surgery and chemotherapy (remained in remission for 13 years)was admitted. Since this time, she recurred locally despite resections and multiple lines of chemotherapy, but without metastasising. She was amputated in 2008.
Conclusion: preoperative chemotherapy is feasible despite low age of the patients, can allow conservative surgery and avoid late metastases.
Correspondence should be addressed to Professor Stefan Bielack, Olgahospital, Klinikum Stuttgart, Bismarkstrasse 8, D-70176 Stuttgart, Germany. Email: s.bielack@klinikum_stuttgart.de
