Publication de Nicole Delepine : Strategy of treatment in Desmoïd tumors (agressive fibromatosis in children and adult to avoid absolutely radiotherapy).

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Publié sur : Page 74, CCOS abstracts in Sarcoma. Ed. Taylor et Francis. Volume 5, n°1 - 2001
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Strategy of treatment in Desmoïd tumors (agressive fibromatosis in children and adult to avoid absolutely radiotherapy).
S. Alkallaf, B. Markowska, H. Cornille, L. Belarbi, G. Delépine, Nicole Delepine

Strategy Of Treatment In Desmoid Tumors (Aggressive Fibromatosis In Children And Adults): To Avoid Absolutely Radiotherapy


Desmoid tumor is a 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 years; range, 1± 70 years). Locations were inferior limb (19), superior limb (17), axial (9), head and neck (5). Seventeen 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 did not expose to heavy functional sequellaes (22). The other patients were treated by large resection, but never invalidant. Fourteen patients received pre- or/and post-operative chemotherapy. Ten received Interferon cc, and nine received tamoxifen.

Results:
Mean follow-up is 5 years 3 months. Thirty-four patients are in complete remission, nine in stable disease, six evolutive disease, and one died with thoracic complications. Following repeated surgery, functional sequellaes are numerous: eight nerve palsies, eight 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 schematising the indications of treatment.
1. If the desmoid tumor does not 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 that could be hurt by surgery and lead to dramatic sequellaes, pre-operative chemotherapy is useful, in order to soften the tumor and to help surgery.

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




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