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 baselineMRI imaging 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 advisedin a multidisciplinary sarcoma tumor board

• Tumor deemed accessible for cryoablation procedure by the operator in a type Icenter. (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 1cm security margin according to assessment by referral center (type 1) forcryoablation

• 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 totreatment initiation (D0)

• Subject able to understand the objectives and risks of the research and to givedated and 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 atscreening/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 othervinca-alkaloids, or other constituents to navelbine, to vinblastine, to methotrexateor any excipients, such as, but not limited to current or recent (within 2 weeks)severe infection, severe renal failure, severe hepatic injury, chronic respiratoryfailure

• Any contraindication including hypersensitivity to gadoteric acid, meglumine or anydrug containing gadolinium

• Concomitant treatment with: yellow fever vaccine, prophylactic treatment withphenytoin, trimethoprim, ciprofloxacin, penicillins, probenecid, acetylsalicylicacid, NSAIDs, PPIs, acitretin, azote protoxide, St John's wort

• Any contra-indication for the procedure as stated by the interventional radiologistin terms of tumor size, proximity to neural/vascular structures or adjacent organsat 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 endpointsof the present study

• 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)