Posters— Pediatric Oncology

STRATEGY OF TREATMENT IN DESMOID TUMORS (AGGRESSIVE FIBROMATOSIS IN CHILDREN AND ADULTS): TO AVOID ABSOLUTELY RADIOTHERAPY

N. Delepine, S. Alkallaf, R Markowska, H. Cornifie, L. Belarbi, G. Delepine- Oncologic Paediatric Unit. Avicenne University Hospital - 125 Bd de Stalingrad - 93009 Bobigny Cedex- France -E.mail: nicole.delepine@avc.ap-hop-paris.fr

Desmoid tumor is an histologic benign tumor. Nevertheless, frequent relapses are able to threaten life or conservation of limb. To point out the best indications of treatment, we reviewed our cases.

From 1981 to 1998, we treated 50 patients with fibromatosis (mean age 28 (1-70), locations were inferior limb (19), superior limb (17), axial (9), head and neck (5).

17 patients were seen at first hand, 33 with relapses. Treatment was adapted to each patient, in function of age, history, and risks of spontaneous evolution.

En bloc extratumoral resection was performed each time, when surgery didn’t expose to heavy functional sequellaes (22). The other patients were treated by large resection, but never invalidant. 14 patients received pre or/and post-operative chemotherapy. 10 received Interferon cc, and 9 tamoxifen.

Results: Mean follow-up is 5 years 3 months. 34 patients are in complete remission, 9 in stable disease, 6 evolutive disease, I died with thoracic complications. Following repeated surgery, functional sequellaes are numerous : 8 nerve palsies, 8 articular stiffnesses. Major functional sequellaes came from surgery with radiotherapy. In one case, radiotherapy of a cervical lesion led to impossibility of surgery and to death. The analysis of our series leads to schematise the indications of treatment:

1. If the desmoid tumor doesn’t threaten life or limb, only surgery is needed as large as possible, avoiding functional sequellaes.
2. When tumoral relapses appear, treatment with Tamoxifen must be discussed.
3. When the tumor invades noble structures which could be shirt by surgery and lead to dramatic sequellaes, preoperative chemotherapy is useful, in order to soften the tumor and to help surgery.

Conclusion: in this unfore see cable illness, backgrounds of indications are respective risks of spontaneous evolution and of treatment. Besides surgery, needed in fast all cases, but often insufficient, it must be considered the value of interferon, tamoxifen and/or chemotherapy. The most important concept in the necessity to adapt a well-balanced treatment avoiding sequellaes and particularly radiotherapy. This last attitude leads to most heavy sequellaes and therapeutic deadlocks.