Desmoid tumor epidemiology and demographics
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Desmoid tumor Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]
Overview
Desmoid tumor accounts for 0.03% of all tumors and less than 3% of all the soft tissue tumors. It affects 1 to 2 per 500,000 people worldwide. Incidence of desmoid tumor is approximately two to four per million population per year in general population. Familial desmoids are present in 10% to 25% of FAP patients. Gender ratio is 2:1 for females to males and commonly affects individuals between 20 to 60 years of age.
Epidemiology and Demographics
- Desmoid tumors are rare
- No significant ethnic or racial predilection
Prevalence
- Desmoid tumor accounts for approximately 0.03 percent of all neoplasms
- It accounts for less than 3% of all the soft tissue tumors
- Affects 1 to 2 per 500,000 people worldwide
- In the United States, 900 to 1,500 new cases are diagnosed per year
- Sporadic desmoid tumors are more common than familial desmoids associated with familial adenomatous polyposis
- Familial desmoid tumor (germline APC mutation) occurs in 10% to 25% of FAP patients[1][2]
- People with germline APC mutation have 850 times greater risk of developing desmoids than the general population[3]
- Sporadic desmoids (APC or the beta-catenin gene mutations) are relatively rare, affect 2 to 5 individuals per million per year in the general population[4][5][6][7]
Incidence
- The incidence of desmoid tumor is approximately two to four per million population per year in general population[8][9]
Gender
- Women of fertile age (especially after childbirth) are more commonly affected with desmoid tumor than men[10]
- Gender ratio is 2:1 for females to males
- Incidence is the same in both genders in children
Age
- Desmoid tumor commonly affects individuals between 20 to 60 years of age[9][11]
- Average age is 30’s to 40’s
- Desmoids are rare in young and elderly
Reference
- ↑ Bertario L, Russo A, Sala P, Eboli M, Giarola M, D'amico F; et al. (2001). "Genotype and phenotype factors as determinants of desmoid tumors in patients with familial adenomatous polyposis". Int J Cancer. 95 (2): 102–7. PMID 11241320.
- ↑ Friedl W, Caspari R, Sengteller M, Uhlhaas S, Lamberti C, Jungck M; et al. (2001). "Can APC mutation analysis contribute to therapeutic decisions in familial adenomatous polyposis? Experience from 680 FAP families". Gut. 48 (4): 515–21. PMC 1728231. PMID 11247896.
- ↑ Ferenc T, Sygut J, Kopczyński J, Mayer M, Latos-Bieleńska A, Dziki A; et al. (2006). "Aggressive fibromatosis (desmoid tumors): definition, occurrence, pathology, diagnostic problems, clinical behavior, genetic background". Pol J Pathol. 57 (1): 5–15. PMID 16739877.
- ↑ Leal RF, Silva PV, Ayrizono Mde L, Fagundes JJ, Amstalden EM, Coy CS (2010). "Desmoid tumor in patients with familial adenomatous polyposis". Arq Gastroenterol. 47 (4): 373–8. PMID 21225148.
- ↑ Fisher C, Thway K (2014). "Aggressive fibromatosis". Pathology. 46 (2): 135–40. doi:10.1097/PAT.0000000000000045. PMID 24378386.
- ↑ Schiessling S, Kihm M, Ganschow P, Kadmon G, Büchler MW, Kadmon M (2013). "Desmoid tumour biology in patients with familial adenomatous polyposis coli". Br J Surg. 100 (5): 694–703. doi:10.1002/bjs.9053. PMID 23334997.
- ↑ Fallen T, Wilson M, Morlan B, Lindor NM (2006). "Desmoid tumors -- a characterization of patients seen at Mayo Clinic 1976-1999". Fam Cancer. 5 (2): 191–4. doi:10.1007/s10689-005-5959-5. PMID 16736290.
- ↑ Reitamo JJ, Häyry P, Nykyri E, Saxén E (1982). "The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the Finnish population". Am J Clin Pathol. 77 (6): 665–73. PMID 7091046.
- ↑ 9.0 9.1 Desmoid tumor. Dr Tim Luijkx and Radswiki et al.Radiopaedia 2015.http://radiopaedia.org/articles/desmoid-tumour. Accessed on January 21, 2016
- ↑ Mankin HJ, Hornicek FJ, Springfield DS (2010). "Extra-abdominal desmoid tumors: a report of 234 cases". J Surg Oncol. 102 (5): 380–4. doi:10.1002/jso.21433. PMID 19877160.
- ↑ Economou, Athanasios; Pitta, Xanthi; Andreadis, Efstathios; Papapavlou, Leonidas; Chrissidis, Thomas (2011). "Desmoid tumor of the abdominal wall: a case report". Journal of Medical Case Reports. 5 (1): 326. doi:10.1186/1752-1947-5-326. ISSN 1752-1947.