Radiotherapy in the Treatment of Aggressive Fibromatosis: Experience From a Single Institution

Desmoid-type fibromatosis is a rare, potentially locally aggressive disease. Herein we present our experience in the treatment with radiotherapy. In total 40 patients who received 44 treatments from 2009 to 2018 at the Heidelberg University Hospital with photons (N = 28) as well as protons (N = 15) and carbon ions (N = 1) were investigated. The median age at radiotherapy was 41 years [range 8-78]. Familial adenomatous polyposis (FAP) was confirmed for nine patients and 30 had a unifocal desmoid tumor. The localizations were abdominal wall, abdominopelvic cavity, thoracic wall, extremity, head and neck and trunk. The median prescribed dose was 54 Gy/ Gy (RBE) [range 39.6-66, IQR 50-60]. Eleven treatments were performed at the time of first diagnosis; 33 at the time of progression or recurrence. Post-operative radiotherapy was performed in 17 cases. The median follow-up time was 32 months [1-153]. At the end of the follow-up interval all patients but one were alive. The estimated local progression free survival of the treated lesion in 3 and 5 years was 76.4% and 63,8%, respectively. The progression-free survival in 3 and 5 years was 72.3 and 58.4% and the overall survival was 97.4 and 97.4%, respectively. In case of macroscopic tumor (N = 31) before radiotherapy a partial remission was observed in 12 cases (38.7%) and a complete remission in 4 cases (12.9%). Progression was observed in 13 (29.5%) cases, predominantly at the margin of the planning target volume (PTV, N = 5, 38,4%) followed by progression within the PTV (N = 4, 30.8%). In univariate analysis multifocal localization was associated with impaired progression-free survival (p = 0.013). One patient developed a grade V gastrointestinal bleeding, otherwise no acute toxicity >°III was observed. Late toxicity was depending on the localization of the desmoid tumor and was especially severe in patients with FAP and abdominopelvine desmoids including gastrointesinal fistula, perforation and abscess. Radiotherapy in the treatment of desmoids can lead to long term control. Treatment of patients with abdominopelvine desmoids should be avoided, as the risk of higher-grade complications is substantial.

Image-guided non-invasive high-intensity focused ultrasound (HIFU) has been gaining recognition in treating musculoskeletal tumors and desmoids. However, there is no consensus on the appropriate perioperative management for patients on ongoing anticoagulation who undergo HIFU ablation. Image-guided HIFU treatment was performed in swine on an ongoing oral anticoagulation protocol (N = 5) in two treatment sessions seven days apart. On day one, a total of twenty locations were ablated, and on day eight, ten more muscle ablations were performed, and the animals were euthanized. Imaging, clinical examination, and histopathology were performed to investigate treated tissue for bleeding. Imaging, clinical examination, and histopathology revealed either no bleeding or, in some samples, only small scattered cavities (0.2-2 mm in diameter) filled with blood. Noninvasive HIFU ablation of muscle may not require a coagulation profile within normal limits.

A total of 14 women and 1 man underwent surgery on desmoid type fibromatosis of the breast. The average patient age was 49 years (range: 22-72 years). The mean tumor size was 2.2 cm (range: 0.8-4.2 cm). The tumor was detectable in mammography in 12 out of 13 patients and in all 15 patients in sonography. MRI was performed preoperatively in 6 patients; in all of the patients, the tumor was visualized with inhomogeneous contrast enhancement. In the imaging procedures, all desmoid type fibromatoses were classified as suspicious. Performing the core biopsy, preoperative histology confirmed desmoid fibromatosis in 12 out of 15 patients. Nuclear stain for beta-catenin was positive in 7 out of 10 patients. Negative staining was found for AE1/A3 in 10 out of 10 and CD34 in 12 out of 12 patients. In all of the patients, a single-stage operation without the detection of border-forming tumor margins was performed. The follow-up interval ranged from 16 to 96 months (mean: 44.86 months, median: 43 months). In this follow-up period, no patient was diagnosed with desmoid tumor recurrence. In imaging, desmoid type fibromatosis of the breast has typical malignancy-related criteria. Extensive preoperative diagnostics enable the planning of complete primary excision of the lesion and reduce the recurrence risk.

Desmoid type fibromatoses has proven to be a diagnostic and therapeutic challenge, as they often appear primarily as a carcinoma of the breast with a high recurrence risk. A digital archive search was performed for the period from 2009 to the end of 2018. Inclusion criteria consisted of histological examination of at least the surgical specimen in the reference pathology department and at least a second opinion diagnosis in the reference radiology department. A total of 14 women and 1 man underwent surgery on desmoid type fibromatosisof the breast. The average patient age was 49 years (range: 22?72 years). The mean tumor size was 2.2 cm (range: 0.8?4.2 cm). The tumor was detectable in mammography in 12 out of 13 patients and in all 15 patients in sonography. MRI was performed preoperatively in 6 patients; in all of the patients, the tumor was visualized with inhomogeneous contrast enhancement. In the imaging procedures, all desmoid type fibromatoses were classified as suspicious. Performing the core biopsy, preoperative histology confirmed desmoid fibromatosis in 12 out of 15 patients. Nuclear stain for beta-catenin was positive in 7 out of 10 patients. Negative staining was found for AE1/A3 in 10 out of 10 and CD34 in 12 out of 12 patients. In all of the patients, a single-stage operation without the detection of border-forming tumor margins was performed. The follow-up interval ranged from 16 to 96 months (mean: 44.86 months, median: 43 months). In this follow-up period, no patient was diagnosed with desmoid tumor recurrence. In imaging, desmoid type fibromatosis of the breast has typical malignancy-related criteria. Extensive preoperative diagnostics enable the planning of complete primary excision of the lesion and reduce the recurrence risk.

Image-guided percutaneous thermal ablation plays an increasingly important role in the multidisciplinary management of musculoskeletal lesions. Established indications for ablation in this setting include the treatment of osteoid osteomas, palliation of painful skeletal metastases, local control of oligometastatic disease, and consolidation of bone tumors at risk for fracture. Emerging indications include the treatment of symptomatic soft tissue masses such as extra-abdominal desmoid tumors and abdominal wall endometriosis. This review will discuss considerations in patient selection and preprocedural workup, ablation technology and techniques, strategies to avoid complications, and expected outcomes of ablation in the musculoskeletal system.

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