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The neck, Thoracic Inlet and Outlet, the Axilla and Chest Wall, the Ribs, Sternum and Clavicles.
Published in Fred W Wright, Radiology of the Chest and Related Conditions, 2022
Although an accompanying Horners syndrome is usually regarded as diagnostic ol malignant spread, and is not seen in patients with nerve damage secondary to irradiation (Kori et al., 1981, Cooke et al., 1988b), this is not always correct. A post-operative Horner's syndrome is often transient. An example caused by aggressive fibromatosis was described by Gebarski et al. (1982).
Oncology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Gill A. Levitt, Penelope Brock, Tanzina Chowdhury, Mark Gaze, Darren Hargrave, Judith Kingston, Antony Michalski, Olga Slater
Aggressive fibromatosis (desmoid tumour) can occur at any site. Intra-abdominal desmoids are more common in adults and could be a feature of mutation in the familial adenomatous polyposis (FAP) gene. FAP should be ruled out when desmoids are multiple or arise in young children. Aggressive fibromatosis is difficult to manage. Complete surgical excision is usually impossible and incomplete resection often provokes regrowth at a more rapid rate than that of the original tumour. Chemotherapy with vinblastine and methotrexate could produce response as well as antioestrogens (tamoxifen or toremifene). Imatinib mesylate, a selective tyrosine kinase inhibitor that targets c-kit, platelet derived growth factor receptor (PDGFR)-α and PDGFR-β has shown some activity in desmoid fibromatosis that is mediated by PDGFR kinase activity.
Skin and soft tissue
Published in Tor Wo Chiu, Stone’s Plastic Surgery Facts, 2018
The name comes from desmos (Greek for band- or tendon-like); the tumour was first described in 1832 by McFarlane and named in 1834 by Müller. The estimated incidence is 1–2 per 500,000. These are firm, irregular tumours arising typically from the muscular aponeurosis of the abdominal wall, especially below the umbilicus. The mean size at presentation is 7 cm. It can be regarded as an aggressive fibromatosis with fibroblast proliferation, variable collagen deposition and mucoid degeneration. It is prone to aggressive local invasion and local recurrence if inadequately excised but is unlikely to metastasise (however, death can still result from the aggressive local invasion). Desmoid tumours have a predilection for parous women in their 30s–50s and have an association with Gardner syndrome. The aetiology is unknown but possible aetiological factors include the following: Trauma – reports of desmoid tumours arising in scars, e.g. Caesarean section and desmoid reported in the capsule surrounding a breast implant.Some say that surgical trauma may stimulate tumour growth particularly in FAP.Hormonal factors – extra-abdominal desmoids may express oestrogen receptors and increased growth is observed during pregnancy, with a peak incidence in post-pubertal and pre-menopausal women. Tumours may regress during menopause, after tamoxifen or combined oral contraceptive (COC) treatment.Genetic factors – Gardner syndrome or FAP with APC gene mutation –desmoid tumours are 1000× more common (incidence of 10%–15%). Familial multicentric fibromatosis – desmoid tumours usually occur singly in non-FAP patients but can occur multifocally without evidence of Gardner syndrome in some patients.
Tumor reduction and symptom relief after electrochemotherapy in a patient with aggressive fibromatosis – a case report
Published in Acta Oncologica, 2018
Joanna Vitfell-Rasmussen, Rikke Mulvad Sandvik, Karin Dahlstrøm, Gina Al-Farra, Anders Krarup-Hansen, Julie Gehl
A 63-year-old woman with FAP and previous colectomy was in December 2012 referred due to severe pain in her right shoulder. An MRI revealed a tumor measuring 7.2 × 2.2 cm in close relation to the right scapula. A biopsy was performed which showed aggressive fibromatosis. Surgical removal was considered to be potentially very extensive, hence the patient began treatment with etodolac 200 mg and exemestane 25 mg daily in March 2013. In August 2013, the MRI showed stable disease, but due to side effects from exemestane, the patient started treatment with letrozole 2.5 mg daily. In June 2014, a Whipple procedure was performed due to high-grade dysplasia in an adenoma in the papilla vateri. As of December 2014, the patient complained about increasing pain in the neck and shoulder and an MRI showed progressive disease. She began treatment with sorafenib 200 mg ×2 daily, but because of side effects, the dose was reduced to 400 mg and 200 mg on alternate days. An MRI in April 2015 showed stable disease, but due to increasing pain, the dose of sorafenib was again increased to 200 mg ×2 daily. The patient was very troubled by diarrhea, exacerbated due to her previous colectomy.
The safety and ablation efficacy of ultrasound-guided high-intensity focused ultrasound ablation for desmoid tumors
Published in International Journal of Hyperthermia, 2021
Rong Zhang, Jin-Yun Chen, Lian Zhang, Ke-Quan Li, Zhi-Bo Xiao, Shao-Jiang Mo, Li Chen, Wen-Zhi Chen
Desmoid tumors (DTs), also known as aggressive fibromatosis (AF) and desmoid-type fibromatosis, are a rare intermediate soft tissue tumor with an incidence of 5.36 new diagnoses per 1 million individuals per year and are characterized by local invasion, non-metastasis, and a variable and often unpredictable clinical course [1–3]. DTs are typically diagnosed in young adults with a peak age of about 30 years and a female predominance [4]. They may develop at any anatomic site and are classified as extra-abdominal, abdominal wall and intra-abdominal types. Although the latter type is often related to familial adenomatous polyposis (FAP)-associated DTs (7.5–16%) with poor prognosis [5,6], most patients can achieve long-term survival [7].