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|LONG TERM SURVIVAL OF CONGENITAL FIBROSARCOMA, ABOUT 3 CASES .|
H. Cornille , S. Alkhallaf , N. Delepine, B. Markowska, G. Delepine
Oncology paediatric unit of University Raymond Poincaré hospital,104 Boulevard R Poincaré , 92380 Garches , France.
EMSOS Congress , Stuttgart, 14/15 May 2009
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 post-operative 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.
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