Traitements des tumeurs desmoides de la racine du mesentere

OBJECTIVE: To elaborate a therapeutic scheme for desmoid tumors developing within the mesenteric root. These uncommon and often unrecognized tumors are difficult to treat as they lie very close to the superior mesenteric vessels. PATIENTS AND METHODS: Retrospective analysis of 14 cases treated in our center over the last 20 years and a review of the literature. RESULTS: The analysis led to the development of a therapeutic scheme. Patients are placed under regular surveillance to detect any progression of the mesenteric root desmoid tumor. In case of progression, surgery should be proposed as often as possible in spite of the real difficulty of this surgery. When the tumor is unresectable, hormone therapy, sulindac, chemotherapy and radiotherapy can be proposed as appropriate. CONCLUSION: Based on the available literature, the proposed decisional tree is a helpful management tool. This scheme should be validated with the help of a National Observatory focusing on this rare disease.