Desmoid tumor epidemiology and demographics
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Desmoid tumor Microchapters |
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Diagnosis |
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Treatment |
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Case Studies |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]
Overview
Desmoid tumor occurs in less than 0.03% of all tumors. The incidence of desmoid tumor is approximately 30 per 100,000 individuals worldwide. Females are more commonly affected with desmoid tumor than males. Desmoid tumor commonly affects individuals between 20 to 40 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]
- 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.