Desmoid Tumor
- PMID: 29083753
- Bookshelf ID: NBK459231
Desmoid Tumor
Excerpt
Desmoid tumors are mesenchymal neoplasms that are considered locally invasive but nonmetastasizing. They are also known as aggressive fibromatosis, deep fibromatosis, and musculoaponeurotic fibromatosis. Even though they do not metastasize, they are locally invasive and cause significant morbidity and mortality. There is no standard approach to treating desmoid tumors; managing them is challenging and requires discussion at a multidisciplinary tumor board.
Despite surgical resection, desmoid tumors have a high rate of local recurrence; accordingly, they have been labeled as intermediate locally aggressive tumors by the World Health Organization (WHO). According to WHO, desmoid tumor is a "clonal fibroblastic proliferation that arises in the deep soft tissues and is characterized by infiltrative growth and a tendency toward local recurrence but an inability to metastasize," even though it may be multifocal in the same limb or body part. Systemic treatment is used in patients where surgery and radiation therapy are either not feasible or will not achieve a cure. Recently, several new drugs have been licensed by the United States Food and Drug Administration (FDA) to treat patients with DT.
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Sections
- Continuing Education Activity
- Introduction
- Etiology
- Epidemiology
- Pathophysiology
- Histopathology
- History and Physical
- Evaluation
- Treatment / Management
- Differential Diagnosis
- Prognosis
- Complications
- Deterrence and Patient Education
- Pearls and Other Issues
- Enhancing Healthcare Team Outcomes
- Review Questions
- References
References
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- PDQ Pediatric Treatment Editorial Board. PDQ Cancer Information Summaries [Internet] National Cancer Institute (US); Bethesda (MD): 2024. Dec 17, Childhood Soft Tissue Sarcoma Treatment (PDQ®): Health Professional Version. - PubMed
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- Wang Z, Wu J, Lv A, Tian X, Hao C. En bloc resection for intra-abdominal/retroperitoneal desmoid-type fibromatosis with adjacent organ involvement: A case series and literature review. Biosci Trends. 2018;12(6):620-626. - PubMed
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- Timbergen MJM, van de Poll-Franse LV, Grünhagen DJ, van der Graaf WT, Sleijfer S, Verhoef C, Husson O. Identification and assessment of health-related quality of life issues in patients with sporadic desmoid-type fibromatosis: a literature review and focus group study. Qual Life Res. 2018 Dec;27(12):3097-3111. - PMC - PubMed
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