Cryoablation Versus Medical Therapy in Desmoid Tumors Progressing After Watchful Waiting

Inclusion Criteria:

• Subject (male or female) with extra-peritoneal desmoid tumor (confirmed by priorbiopsy by an experienced pathologist within the RRePS network)
• 13 years of age or older
• Measurable viable tumor (according to the mRECIST criteria using MRI. The baseline MRIimaging is mandatory in the 2 months prior to treatment initiation (D0).
• Progressive disease (according to the mRECIST criteria) after the watchful waitingperiod or significant increase in symptoms requiring an active therapy, as advised ina multidisciplinary sarcoma tumor board
• Tumor deemed accessible for cryoablation procedure by the operator in a type I center. (In pediatric cases, a careful site tumor analysis will be performed withinterventional radiologist to ensure for limited consequence of cryoablation inpediatric patients especially regarding growth plates).
• 100 % of destruction of the tumor achievable in one procedure of cryoablation with 1cmsecurity margin according to assessment by referral center (type 1) for cryoablation
• ECOG performance status 0-2 at inclusion visit
• Biological and hematological parameters (neutrophils ≥ 1,5.109/L ; platelet count ≥ 100.109/L ; no significant hemostatic abnormalities) in the 4 weeks prior to treatmentinitiation (D0)
• Subject able to understand the objectives and risks of the research and to give datedand signed informed consent. For minors, the consent of the 2 parents must beobtained.
• Subject affiliated to a social health insurance plan
• For a woman of childbearing age: negative blood pregnancy test at screening/inclusionvisit
• Subject agreeing to use a contraceptive method:

Exclusion criteria:

• Intra-peritoneal desmoid tumor
• Known hypersensitivity to vinorelbine or other vinca-alkaloids, or other constituentsto navelbine, to vinblastine, to methotrexate or any excipients, current or recent (within 2 weeks) severe infection, severe renal failure, severe hepatic injury,chronic respiratory failure, concomitant treatment with: yellow fever vaccine,prophylactic treatment with phenytoin, trimethoprim, probenecid, acetylsalicylic acid,phenylbutazone.
• Any contra-indication for the procedure as stated by the interventional radiologist interms of tumor size, proximity to neural/vascular structures or adjacent organs atrisk making the procedure at unacceptable risk
• Impaired hemostasis, that may interfere with the conduct of the cryoablation
• Concurrent participation in other experimental studies that could affect endpoints ofthe present study
• Concurrent use of any antitumor agent or NSAIDs, penicillins, PPIs, acitretin,ciprofloxacin, azote protoxide
• Contraindication to any form of sedation
• Hypersensitivity to gadoteric acid, meglumine or any drug containing gadolinium
• Others contra-indications to MRI
• Pregnancy or breastfeeding
• Impossibility to give the subject informed information (subject in an emergencysituation, patient with comprehension difficulties ...)
• Psychiatric disorders
• Incompetent subject (subject to a legal protection measure: curatorship, guardianship,future protection mandate, family habilitation)