Cryoablation Versus Medical Therapy in Desmoid Tumors Progressing After Watchful Waiting

Inclusion Criteria:

* Subject (male or female) with extra-peritoneal desmoid tumor (confirmed by prior biopsy 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 MRI imaging is mandatory in the 2 months prior to treatment initiation (D0).
* Progressive disease (according to the mRECIST criteria) after the watchful waiting period or significant increase in symptoms requiring an active therapy, as advised in a 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 with interventional radiologist to ensure for limited consequence of cryoablation in pediatric patients especially regarding growth plates).
* 100 % of destruction of the tumor achievable in one procedure of cryoablation with 1cm security 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 treatment initiation (D0)
* Subject able to understand the objectives and risks of the research and to give dated and signed informed consent. For minors, the consent of the 2 parents must be obtained.
* Subject affiliated to a social health insurance plan
* For a woman of childbearing age: negative blood pregnancy test at screening/inclusion visit
* Subject agreeing to use a contraceptive method

Exclusion criteria:

* Intra-peritoneal or multifocal desmoid tumor
* Concurrent or prior use of any antitumor agent for the current desmoid tumor
* Relapse after surgery for desmoid tumor
* Any contraindication including known hypersensitivity to vinorelbine or other vinca-alkaloids, or other constituents to navelbine, to vinblastine, to methotrexate or any excipients, such as, but not limited to current or recent (within 2 weeks) severe infection, severe renal failure, severe hepatic injury, chronic respiratory failure
* Any contraindication including hypersensitivity to gadoteric acid, meglumine or any drug containing gadolinium
* Concomitant treatment with: yellow fever vaccine, prophylactic treatment with phenytoin, trimethoprim, ciprofloxacin, penicillins, probenecid, acetylsalicylic acid, NSAIDs, PPIs, acitretin, azote protoxide, St John's wort
* Any contra-indication for the procedure as stated by the interventional radiologist in terms of tumor size, proximity to neural/vascular structures or adjacent organs at risk making the procedure at unacceptable risk
* Impaired hemostasis, that may interfere with the conduct of the cryoablation
* Contraindication to any form of sedation
* Others contra-indications to MRI
* Pregnancy or breastfeeding
* Concurrent participation in other experimental studies that could affect endpoints of the present study
* Impossibility to give the subject informed information (subject in an emergency situation, patient with comprehension difficulties ...)
* Psychiatric disorders
* Incompetent subject (subject to a legal protection measure: curatorship, guardianship, future protection mandate, family habilitation)