Desmoid Type Fibromatosis of the Breast: Ten-Year Institutional Results of Imaging, Histopathology, and Surgery (ENTIRE ARTICLE)

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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It is challenging to know at the first which patients with desmoid fibromatosis (DF) are better suited to conservative or aggressive treatment. To investigate whether the low signal intensity bundles on T1- or T2-weighted images (WI), termed the “black fiber sign (BFS),” can predict non-progressive behavior in the conservative approach This retrospective study included 59 patients with primary DF managed with wait-and-see approach from 2005 to 2018 and serial MR images were analyzed. Three observers blinded to the patient information verified the presence or absence of BFS on baseline T1 or T2WI. The likelihood of progression-free survival (PFS) after ascertaining the presence or absence of the BFS was estimated using the Kaplan-Meier method and analyzed with the log-rank test. PFS was significantly higher in cases with BFS than without BFS on T1WI (p??0.8). Our retrospective observational data support that presence of BFS in baseline MRI may be a predictor for progression-free survival of DF. BFS on T1WI is easily identifiable and can be utilized clinically in patients with DF. We proposed a new imaging marker for prediction of desmoid fibromatosis progression. The absence of black fiber sign predicted a high risk of disease progression.

Patients with familial adenomatous polyposis (FAP) may undergo either ileorectal anastomosis (IRA) or ileal pouch anal anastomosis (IPAA) depending on the degree of rectal involvement. Desmoid tumors (DTs) may arise postoperatively. Whether IPAA is associated with a higher risk of DTs as compared with IRA remains controversial. The purpose of this study was to determine whether IPAA increased the risk of DTs by analyzing the published data that compared IRA and IPAA as the primary treatment for FAP. A metaanalysis was performed to analyze the published data between 1989 and 2019. IRA and IPAA were compared with respect to the incidence of DTs. Eight retrospective studies with a total of 1072 patients were identified: 491 underwent IPAA and 581 IRA. There was no significant difference in the incidence of DTs between IPAA and IRA (11.81% vs. 9.47%, OR 0.95, P?=?0.85). Meanwhile, the overall complication (42.97% vs. 36.76%, OR 1.32, P?=?0.11), incidence of cancer (4.88% vs. 8.37%, OR 0.28, P?=?0.26), and overall mortality (0.33% vs. 5.20%, OR 0.49, P?=?0.53) were comparable too. Ileoanal pouch surgery is associated with similar risk of desmoid in patients with FAP after surgery.

The aim of this study was to identify predictors of desmoid progression during observation. Primary or recurrent desmoid patients were identified retrospectively from an institutional database. In those managed with active observation who underwent serial magnetic resonance imaging (MRIs) with T2-weighted sequences, baseline tumor size was recorded, and 2 radiologists independently estimated the percentage of tumor volume showing hyperintense T2 signal at baseline. Associations of clinical or radiographic characteristics with progression-free survival (PFS; by RECIST) were evaluated by Cox regression and Kaplan-Meier statistics. Although PFS was not associated with size, site, or age, it was strongly associated with hyperintense T2 signal in ≥90% versus <90% of baseline tumor volume (as defined by the “test” radiologist; hazard ratio = 11.3, P = 0.003). For patients in the ≥90% group (n = 20), 1-year PFS was 55%, compared with 94% in the <90% group (n = 17). The percent tumor volume characterized by hyperintense T2 signal is associated with desmoid progression during observation and may help distinguish patients who would benefit from early intervention from those who may be reliably observed.