Use of High-intensity Focused Ultrasound in the Management of Extra-abdominal Desmoid Tumors

High-intensity focused ultrasound ablation is an emerging mode of therapy for benign and malignant neoplastic processes; with respect to musculoskeletal tumors, high-intensity focused ultrasound ablation has been used for palliative treatment of bone metastases and primary treatment of osteoid osteoma, synovial sarcoma, osteosarcoma, Ewing sarcoma, chondrosarcoma and giant cell bone cancer. Experience with high-intensity focused ultrasound ablation of extra-abdominal desmoids is limited and in particular it has been reported by Wang et al. in 2011 and by Avedian et al. in 2015. According to these research groups, high-intensity focused ultrasound is a safe and effective non-invasive therapeutic modality in the treatment of desmoid tumors. High-intensity focused ultrasound may be useful to treat soft tissue tumors of extremities, especially in situations in which standard treatments would be associated with unacceptable morbidity as is often the case with extra-abdominal desmoids.

This study aims to evaluate the safety and effectiveness of en bloc resection in intra-abdominal/ retroperitoneal desmoid-type fibromatosis (IA/RPDF) with adjacent organ involvement. This retrospective clinical study included 21 patients who were diagnosed with IA/RPDF and underwent tumor resection at a single center between March 2013 and June 2018. All patients included in the study underwent surgery with curative intent, and IA/RPDF with adhesive organs was removed en bloc. The safety of surgical treatment was verified by the analysis of intraoperative bleeding, postoperative morbidity and perioperative mortality. The efficacy of surgical treatment was evaluated based on the status of tumor infiltration of adjacent organs and patient follow-up results. Complete macroscopic (R0 or R1) resection was achieved in all cases. For postoperative pathological diagnosis at our center, tumor infiltrated at least one organ in each patient. Infiltration was noted in 45 resected organs (45/57, 78.9%). Grade III-V postoperative morbidity developed in one patient (4.8%). During the follow-up, one patient developed local recurrence. No DF-related death was noted during the follow-up. The 3-year disease-free survival rate was 94.1% (95% confidence interval: 83.6-100%). Therefore, en bloc resection of the tumor and involved adjacent organs is a safe and effective treatment modality for IA/RPDF.

Our aim was to examine the outcome of radiotherapy (RT) in the treatment of primary or recurrent unresectable desmoid tumors of the neck. A retrospective analysis was performed on 30 patients between 1/2008 and 12/2017, with 3 primary and 27 recurrent unresectable desmoid tumors of the neck. The median follow-up time was 47.5 months (range 2-126 months). Radiotherapy dose varied from 50 to 66Gy (median 60 Gy, 23/30 patients) with all fraction size of 2 Gy. The objective response rate (ORR: CR or PR) to definitive RT was 56.7% (17/30 patients). Three patients developed local recurrence after RT, which occurred out-of-target. On univariate analysis, ORR was significantly influenced by tumor size (＜5 cm vs. >=5 cm) (p = 0.023). Age ( 40 vs. >40 years) (p = 0.804), gender (p = 0.629) and RT dose (<60, =60 vs. >60 Gy) (p = 0.613) were not significantly associated with ORR. The most common acute side effect of the radiation-related complication was grade 1-2 skin toxicity. Radiotherapy is a valuable option in the management of primary or recurrent unresectable desmoid tumors of the neck when surgical resection is not feasible. According to our results, radiotherapy is especially beneficial in tumor size ＜5cm. The complications of the radiation-related are acceptable.

We present the case of a female patient of 30 years old with a history of bronchial asthma, which was studied in 2011 for abdominal pain, gastric fullness, and increased abdominal perimeter. A large tumor mass was diagnosed that involved abdominal organs and large vessels. In the case of retroperitoneal fibromatosis, surgery was performed twice, without resecting the tumor. With the confirmation by biopsy of an aggressive fibromatosis, it´s proposed by Oncology Service (January 2012), the use of chemotherapy with anthracyclines in neo-adjuvant setting, obtaining an objective response at the end of six cycles, achieving the resection of 80% of the tumor. Subsequently, she remained with hormone therapy (tamoxifen-20mg/day), and at the end of 4 months, another surgical intervention was performed, obtaining a complete resection of the residual tumor disease. It is maintained to date, in follow-up consultation by Oncology, with a satisfactory evolution, asymptomatic, and without clinical or imaging evidence of fibromatosis.

The objective of this study is to present the clinical outcomes of patients with Extra-abdominal desmoid tumor (EDT) treated surgically between 1995 and 2016. A total of 223 medical records with histopathological reports were evaluated. Only 23 cases of EDT were included in the present study. Twelve (52.2%) patients had the tumor on the lower limbs, seven (30.4%) on the upper limbs and four (17.4%) cases were reported on the back. Five (21.7%) patients had tumors measuring less than 5 cm, while eighteen (78.3%) patients had tumors measuring more than 5 cm. All patients underwent surgery as the definitive treatment in our institute. Twelve (52.2%) cases presented negative margins (NM) and eleven (47.8%) cases had positive margins (PM). Local recurrence (LR) occurred in eleven (47.8%) patients. Impairment of the surgical margin was the only prognostic factor found for LR of EDT. Level of Evidence IV, Case Series.

Cryotherapy is increasingly used and has been included in guidelines for selected tumor entities such as renal cell carcinoma. Cryo-immunotherapy and combination treatments are future areas of interest.

Cryoabalation may be used in many indications. Its major advantages are its unique visualization and the anesthesiologic effects of cold. While there are only a few prospectively randomized trials, the existing data on the use of cryoablation is promising. Its use appears to be justified in selected tumors, oligometastatic patients and for palliative indications.