Long-term Efficacy of Methotrexate Plus Vinblastine/Vinorelbine in a Large Series of Patients Affected by Desmoid-Type Fibromatosis.

We retrospectively reviewed data from all adult patients with sporadic desmoid-type fibromatosis treated with MTX and vinca alkaloids at our institution between 1989 and 2014. In this series, chemotherapy with MTX and vinca alkaloids is confirmed to be active and effective, with a remarkable PFS, higher in responding patients, and limited toxicity. Even progression can be successfully rechallenged.

This review focuses on sarcoma subtypes that frequently cause these complications: bony and soft tissue sarcomas leading to limb salvage or amputation, desmoid tumors, and malignant peripheral nerve sheath tumors. Rehabilitation approaches and outcomes are discussed, as well as considerations for childhood sarcoma survivors transitioning to adulthood.

Data were retrospectively collected from 17 children/adolescents treated for sporadic desmoid tumors at a tertiary pediatric hospital in 1988-2016. After a median follow-up of 3.3years, 10 patients were alive in complete remission, 5 had stable disease, and 2 had reduced tumor size. Five-year overall survival was 100%, and event-free survival, 87.5%.Pediatric desmoid tumors are rare, with variable biologic behavior and morbidity. Treatment requires a multidisciplinary approach.

Since December 2014, 14 magnetic resonance-guided focused ultrasound (MRgFUS) treatments for desmoid tumors were performed at our institution in seven patients. MRgFUS is an effective treatment for desmoid tumors of the thigh with a favorable side effect profile, allowing for repeated treatments if necessary. Ablation size and safety can be improved with far-field coupling devices, careful patient positioning, and optimized sonication planning.

Several reports of desmoid tumors arising in the vicinity of the fibrous capsule of a breast implant have been described, but to date, the authors are not aware of any published cases following autologous breast reconstruction. This report describes a desmoid tumor developing after a muscle-sparing free transverse rectus abdominis musculocutaneous (TRAM) flap for breast reconstruction and subsequent surgical management.

There is no report from Iran about the treatment of this tumor (aggressive fibromatosis), and all published articles are single-case reports, therefore in this study, we report our experience from two of the largest referral centers of the South of Iran. During five years (2007-2011), among more than 20000 surgical pathology specimens, 25 cases of fibromatosis were identified. Clinicopathologic findings of desmoid tumor in Iran are very similar to other countries, however, there is still much controversy about the method of treatment for fibromatosis, and there are many challenges for patients, regarding multiple episodes of recurrence and the infiltrative aggressive nature of fibromatosis.