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Medical condition
Aggressive fibromatosis
Other names
Desmoid tumor, deep fibromatosis, desmoid fibromatosis
Desmoid tumor as seen on CT scan
Specialty
Oncology, surgery, radiology
Complications
Pain, loss of function, restricted movement
Usual onset
30–40 years[1]
Risk factors
CTNNB1 and APC gene mutations, familial adenomatous polyposis, estrogen levels, pregnancy, physical trauma or surgery
Diagnostic method
Biopsy
Differential diagnosis
Broad, including fibroblastic sarcomas, superficial fibromatosis, nodular fasciitis, gastrointestinal stromal tumor, and scar tissue
Treatment
Watchful waiting; surgery; radiation therapy‚ chemotherapy; antiestrogen medication; NSAIDs; ablation with cold, heat, or ultrasound
Incidence
5–6 per million per year[2]
Aggressive fibromatosis or desmoid tumor is a rare condition. Desmoid tumors are a type of fibromatosis and related to sarcoma, though without the ability to spread throughout the body (metastasize). The tumors arise from cells called fibroblasts, which are found throughout the body and provide structural support, protection to the vital organs, and play a critical role in wound healing. These tumors tend to occur in women in their thirties, but can occur in anyone at any age. They can be either relatively slow-growing or malignant. However, aggressive fibromatosis is locally aggressive and can cause life-threatening problems or even death when the tumors compress vital organs such as intestines, kidneys, lungs, blood vessels, or nerves. The condition is rarely fatal. Most cases are sporadic, but some are associated with familial adenomatous polyposis (FAP). Approximately 10% of individuals with Gardner's syndrome, a type of FAP with extracolonic features, have desmoid tumors.[3]
The World Health Organization reclassified desmoid tumors (termed desmoid-type fibromatosis) as a specific type of tumor in the category of intermediate (locally aggressive) fibroblastic and myofibroblastic tumors.[4]
Histologically they resemble very low-grade fibrosarcomas,[5] but they are very locally aggressive and tend to recur even after complete resection. The condition is "characterized by a variable and often unpredictable clinical course."[2] There is a tendency for recurrence in the setting of prior surgery; in one study, two-thirds of patients with desmoid tumors had a history of prior abdominal surgery.[6] The condition can be chronic and may be debilitating.[7]
^Master SR, Mangla A, Puckett Y, Shah C (January 2023). "Desmoid Tumor". StatPearls. Treasure Island: StatPearls Publishing. PMID 29083753. Archived from the original on 2023-06-07. Retrieved 2023-08-14 – via National Institutes of Health.
^ abCite error: The named reference europeanconsensus was invoked but never defined (see the help page).
^Nieuwenhuis MH, De Vos Tot Nederveen Cappel W, Botma A, Nagengast FM, Kleibeuker JH, Mathus-Vliegen EM, et al. (February 2008). "Desmoid tumors in a dutch cohort of patients with familial adenomatous polyposis". Clinical Gastroenterology and Hepatology. 6 (2): 215–219. doi:10.1016/j.cgh.2007.11.011. PMID 18237870. S2CID 26052046.
^Sbaraglia M, Bellan E, Dei Tos AP (April 2021). "The 2020 WHO Classification of Soft Tissue Tumours: news and perspectives". Pathologica. 113 (2): 70–84. doi:10.32074/1591-951X-213. PMC 8167394. PMID 33179614.
^"desmoid" at Dorland's Medical Dictionary
^Lynch HT, Fitzgibbons R (December 1996). "Surgery, desmoid tumors, and familial adenomatous polyposis: case report and literature review". The American Journal of Gastroenterology. 91 (12): 2598–2601. PMID 8946994.
^Valesano JC, Schmitz JJ, Jensen NM, Schultz GR, Callstrom MR (December 2017). "Desmoid Tumors: A Review of Their Natural History, Imaging, and Treatment". Journal of Radiology Nursing. 36 (4): 211–217. doi:10.1016/j.jradnu.2017.09.003.
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