Desmoid fibromatosis associated with Endobutton use for anterior cruciate ligament reconstruction

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Purpose: To retrospectively review the various methods used to treat extra-abdominal desmoid fibromatosis (EDF) at our institution to compare treatment response and complications with those for the emerging option of percutaneous cryoablation therapy. Methods: A single-center retrospective review was conducted to identify patients with EDF who underwent some form of treatment for EDF in any combination (including medical therapy, surgery, percutaneous ablation and radiation therapy) at our institution between January 2007 and January 2020. Patients with pathological evidence of EDF and pretreatment and posttreatment images were included. Medical records and imaging data were also reviewed. Treatment response assessment was based on tumor size on follow-up imaging. Results: A total of 41 patients (30 women; mean age, 34 y; range, 18-79 y) were included in the study. The extremities (44%) and back (22%) were the most common locations of EDF. Patients underwent a variety of treatment methods, including medical therapy (31 patients), surgery (24 patients), cryoablation (7 patients), radiation therapy (6 patients), and radiofrequency ablation (4 patients). Reduction in lesion size after at least 3 months of follow-up was most common in patients who underwent surgery alone (5 patients) or cryoablation (4 patients). Among all study patients, there were 10 minor complications and 3 major complications. Complication rates were lowest in patients who underwent cryoablation (no complications). Conclusion: Although further work is needed, the early data in this study offers promising results regarding the clinical application of cryoablation for EDF, which appears safer than radiofrequency ablation and a potentially effective.

Introduction: Desmoid tumor (DT) of the abdomen is a challenging and rare disease. The level of evidence available to document their treatment is relatively low, however, recent publications of prospective studies have allowed to precise their management. Methods: This document is a summary of the French intergroup guidelines realized by all French medical and surgical societies involved in the management of DT located in the abdomen or associated with adenomatous polyposis. Recommendations are graded in four categories (A, B, C and D), according to the level of evidence found in the literature until January 2021. Results: When the diagnosis of DT is suspected a percutaneous biopsy should be performed when possible. A molecular analysis looking for pathogenic mutations of the CTNNB1 and APC genes should be systematically performed. When a somatic pathogenic variant of the APC gene is present, an intestinal polyposis should be searched. Due to a high rate of spontaneous regression, non-complicated DT should first benefit from an active surveillance with MRI within 2 months after diagnosis to assess the dynamic of tumor growth. The treatment decision must be discussed in an expert center, favoring the less toxic treatments which can include broad spectrum tyrosine kinase inhibitor or conventional chemotherapy (methotrexate-vinblastine). Surgery, outside the context of emergency, should only be considered for favorable location in an expert center. Conclusion: French guidelines for DT management were elaborated to help offering the best personalized therapeutic strategy in daily clinical practice as the DT therapeutic landscape is complexifying. Each individual case must be discussed within a multidisciplinary expert team.

Introduction Desmoid tumors are benign mesenchymal tumors developed at the expense of muscular fasciae and aponeuroses. The mammary localization is a rare entity, representing less than 0.2% of all breast tumors. It is characterized by a strictly local evolution and its tendency to recur without giving metastases. Its clinical and radiological presentation is similar to a breast carcinoma which is the main differential diagnosis. Case presentation Patient aged 51 years, primigravida primiparous, followed for right breast cancer diagnosed at the age of 49 years for which she received a right mastectomy and axillary lymphnode dissection and contralateral breast reduction. It was a 4 cm infiltrating ductal carcinoma, SBR III Luminal B, 0 N+/20 N with presence of fibrous mastopathy without sign of malignancy at the left breast reduction specimen.The patient received adjuvant chemotherapy, external radiotherapy and hormone therapy.One year after surgery, the patient returned for a four x 2 cm left breast nodule in the upper medial quadrant. The biopsy confirmed the diagnosis of fibromatosis of the breast. A wide local excision was performed. Discussion The etiology of this tumor is unknown, however, physical, hormonal and genetic factors play an important role in the development of desmoid tumor.The clinical presentation is similar to breast carcinoma, making it difficult to differentiate this tumor from breast carcinoma. Breast imaging techniques are not specific for desmoid fibromatosis. Treatment is based primarily on complete surgical excision. Conclusion Breast fibromatosis is a rare entity, clinically and radiologically mimicking breast cancer. Only histology will provide the diagnosis. The treatment of choice is based on complete surgical excision with healthy safety margins.

A 39-year-old woman presented to the Emergency Department with dysuria and persistent abdominal pain for several weeks. The pain increased with palpation. Physical examination was otherwise normal. Computerized tomography (CT) scan revealed a round structure of unknown origin against the caecum and terminal ilium suspect for a GIST tumor and a fluid-filled right uterine tube suggestive of pyosalpinx. The following day a laparoscopic surgery with removal of the pyosalpinx and complete right hemi-colon including the suspected lesion was performed and biopsies of the surrounding peritoneum, meso and ileum were taken. Immunohistochemical tests (CD117/DOG1) and molecular analysis (KIT/PDGFRA) ruled out GIST. SMA and desmin were also negative. β-catenin along with next generation sequencing which revealed a likely pathogenic mutation in exon 3 of the CTNNB1 gene, suggested a diagnosis of desmoid-type fibromatosis (DF). Biopsies of the lymph nodes were negative.

Background: Desmoid fibromatosis (DF) is a rare clonal proliferation of fibroblasts and myofibroblasts. It develops in the connective tissues and does not metastasize but may infiltrate adjacent structures. Because of the rarity of these tumors and the unpredictable natural history of the disease, well-defined and precise guidelines of the optimal treatment for DF have not been formulated. Case presentation: Here, we present a giant abdominal DF that invaded the right spermatic cord and iliac vessels. The lesion was excised with external iliac artery dissection; however, the vein was sacrificed. The abdominal wall defect was then repaired with a polypropylene mesh. The lesional cells are positive for β-catenin. Conclusions: In the past decades, there has been a change in the treatment of DF. The “wait and see” policy has been considered initially in most cases. Surgical intervention remains a valid option for symptomatic lesions. The optimal regimes of the tumor should not take the risk of making the patient more symptomatic than the lesion itself.