Place des thérapeutiques médicales dans la prise en charge des tumeurs desmoïdes de l'enfant

Aggressive fibromatoses (desmoids tumors) are a rare nonmetastasizing fibrous tumor characterized by a local invasive capacity, a high recurrence rate after excision. Surgery remains the standard treatment. Wide excision with negative margins seems to decrease the chance of recurrence but must be not mutilating. Adjuvant high dose radiotherapy may have a good result but must be employed in selected patients. Drug therapy, essentially with continuous low dose adjuvant chemotherapy using methotrexate and vinblastine may be a reasonable choice, with low toxicity. Non-cytotoxic drug therapy represented by antiestrogens and non-steroidal anti-inflammatory drugs may have a beneficial effect. Antiproliferative effects of interferon and other drugs such as retinoïc acid have shown an encouraging response rates. The exactly role of this therapies remains controversial. Cellular, molecular and genetic investigations may be helpful to understand the mechanism of desmoid tumor proliferation. The aim of this study is to discuss the place of different medical therapies in the treatment of desmoid tumors, with review of literature.