Difficulties in recruitment.

The goal of this observational study is to learn about the effects of Electrochemotherapy (ECT) in patients with Desmoid Fibromatosis. The main question it aims to answer is:

Is ECT effective in reducing the size of the lesion and improving patients' symptomatology?

Patients will be followed with MRI and Quality of Life questionnaires at 3, 6 and 12 months after the intervention.

Desmoid fibromatoses are rare and locally aggressive, characterized histologically by monoclonal myoblasts present in abundant stromal tissue.The current therapeutic strategy has abandoned primary resection, as recurrences after resection are common and often their phenotype is more infiltrative. Nonsurgical approaches remain suboptimal. For asymptomatic disease, current guidelines suggest an initial period of active surveillance.

Other minimally invasive local treatments such as cryoablation have been shown to be effective; however, certain patients are not candidates for such treatment because of lesion location/characteristics.

An additional minimally invasive treatment with currently little evidence in the literature is arterial embolization. However, this procedure cannot be performed in the case of superficial lesions, nor in the presence of shared vasculature between the lesion and other healthy tissues or organs. In the absence of other treatments in use, electrochemotherapy treatment can be performed, which has proven effective on several other types of soft tissue and bone tumors, malignant or benign, treated at our Institute.

Electrochemotherapy is based on the principle whereby reversible electroporation causes the application of short, high-voltage electrical pulses to temporarily align water molecules on the hydrolipid layer of the cell membrane, leading to a reversible local increase in permeability by increasing the intracellular concentration of Bleomycin and its toxicity on tumor cells by a factor of up to several thousand.

Inclusion Criteria:

• Symptomatic Desmoid Fibromatosis and in active phase (documented growth at last follow-up)
• Patients not eligible for surgery, cryoablation, or embolization
• Signature of study-specific informed consent.

Exclusion Criteria:

• Patients with fibromatosis not in active phase, documented clinically and with imaging investigations (MRI, CT)
• Patients with documented active infection
• Previous treatment for < 30 days
• Pregnancy and lactation status
• Patients of childbearing age without contraceptive use for at least 3 months
• Presence of metal synthetic media at the site of treatment
• COPD with FiO2 < 30 mmHg
• Impaired renal function with eGFR

Patients with diagnosis of desmoid fibromatosis with an indication for electrochemotherapy treatment

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