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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).
Follow-up protocols in peritoneal malignancy
Published in Tom Cecil, John Bunni, Akash Mehta, A Practical Guide to Peritoneal Malignancy, 2019
Anuradha Chandramohan, Sourav Panda, Nehal Shah
Intra-abdominal fibromatosis, or desmoid tumour, is an unusual mesenchymal mass that can be locally aggressive but without metastatic potential. It may simulate peritoneal recurrence on imaging surveillance and can be difficult to differentiate. Even with serial imaging and PET/CT, the interval growth and moderate FDG avidity can mimic recurrence. Fibromatosis can present as extra-abdominal (60%), abdominal wall (25%) or intra-abdominal (8%–15%) masses. Intra-abdominal fibromatosis usually arises in the mesentery, retroperitoneum or the omentum. Although fibromatosis does not metastasize, the tumour has the potential to locally invade abdominal and pelvic organs with local recurrence rates up to 77% [30,31]. Figures 13.5 through 13.8 show different cases that turned out to be fibromatosis, emphasizing the difficulty in diagnosis and the need to warn patients that sometimes masses are not malignant.
The skin
Published in Angus Clarke, Alex Murray, Julian Sampson, Harper's Practical Genetic Counselling, 2019
Congenital fibromatosis, also known as congenital generalised fibromatosis, is a rare disorder in which multiple spindle-cell fibromatous tumours occur and commonly mature spontaneously. It has several features in common with hyaline fibromatosis syndrome, but the genes implicated are different. The condition may be fatal if gut tumours occur but is usually benign. Although once considered to show autosomal recessive inheritance, autosomal dominant inheritance with incomplete penetrance (especially in older individuals) is now considered much more probable with at least two loci implicated. Careful search for small lesions in apparently unaffected parents is important.
Lipofibromatosis
Published in Fetal and Pediatric Pathology, 2019
Lipofibromatosis is a distinctive benign pediatric soft tissue tumor first described by Fetsch et al. in 2000 [1]. Previously, it was designated as infantile/juvenile fibromatosis. The entity was included in the WHO blue book in the year 2002 under the category of fibroblastic/myofibroblastic tumors. An update on current WHO has reclassified it under intermediate/locally aggressive fibroblastic/myofibroblastic tumors. Lipofibromatosis is defined as a benign pediatric tumor with abundant, usually mature, adipose tissue admixed with a spindle cell (fibromatosis-like) component, often concentrated in septal and perimysial locations [2]. It should be differentiated from the various other benign fibrous and adipocytic tumors of childhood including fibrous hamartoma of infancy, calcifying aponeurotic fibroma, lipoblastoma, intramuscular lipoma, and lipofibromatosis-like neural tumor. Although benign, the tumor is notorious for its high rate of recurrence, variably reported from 33% to 72% in the literature [1, 3]. The diagnosis is mainly pathological as the radiological and clinical features are nonspecific. The histopathology is the mainstay and the ancillary techniques have little role in the final diagnosis. We report a case of lipofibromatosis on the upper back of an infant with a discussion of its clinicopathological features and histopathological clues to differentiate it from other fibrous and adipocytic tumors of the infancy and childhood.
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.
What’s the latest with investigational drugs for soft tissue sarcoma?
Published in Expert Opinion on Investigational Drugs, 2022
Elena Cojocaru, Andrea Napolitano, Cyril Fisher, Paul Huang, Robin L Jones, Khin Thway
Although desmoid-type fibromatosis is not capable of metastasis and is not a malignant tumor per se, it is an infiltrative, locally aggressive neoplasm with a propensity for local recurrence, and is usually treated in a center with expertise in STS. The treatment of desmoid-type fibromatosis is often complex due to the indolent but locally aggressive nature of these tumors. The current recommendations are to initially observe the tumoral evolution, then intervene with oral treatments, often low-dose chemotherapy or tyrosine kinase inhibitors, if rapid progression or symptomatic disease [103,104]. Pathogenesis of desmoid tumors is based on the inappropriate activation of the Wnt/beta-catenin signaling pathway [105]. It was demonstrated that Notch signaling counteracts the Wnt/beta-catenin pathway in colorectal cancer, due to crosstalk between the two pathways [106]. Therefore, the Notch pathway became an attractive target in the treatment of aggressive desmoid fibromatosis [107]. Nirogacestat is a gamma-secretase inhibitor which has the ability to bind to gamma-secretase, blocking proteolytic activation of Notch receptors. The phase 1 study with nirogacestat in patients with desmoid fibromatosis, demonstrated an acceptable toxicity profile and showed objective responses in five of seven patients, with a median treatment duration of 49.5 months [108]. A subsequent phase 2 study, enrolling heavily pre-treated patients with desmoid fibromatosis, showed an overall response rate of 29% [109]. The phase 3 trial of nirogacestat versus placebo, has completed accrual after 142 subjects with desmoid-type fibromatosis were enrolled and the results are expected to be presented in the first half of 2022 (NCT03785964).