Desmoid tumor epidemiology and demographics: Difference between revisions
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==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
*Desmoid tumors are rare | *[[Desmoid tumor|Desmoid tumors]] are rare | ||
*No significant ethnic or racial predilection | *No significant [[Ethnic group|ethnic]] or [[racial]] predilection | ||
===Prevalence=== | ===Prevalence=== | ||
*Desmoid tumor accounts for approximately 0.03 percent of all neoplasms | *[[Desmoid tumor]] accounts for approximately 0.03 percent of all [[neoplasms]] | ||
*It accounts for less than 3% of all the soft tissue tumors | *It accounts for less than 3% of all the [[soft tissue]] [[tumors]] | ||
*Affects 1 to 2 per 500,000 people worldwide | *Affects 1 to 2 per 500,000 people worldwide | ||
*In the United States, 900 to 1,500 new cases are diagnosed per year | *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 | *Sporadic [[Desmoid tumor|desmoid tumors]] are more common than [[familial]] [[Desmoid tumor|desmoids]] associated with [[familial adenomatous polyposis]] | ||
*Familial desmoid tumor (germline APC mutation) occurs in 10% to 25% of FAP patients<ref name="pmid11241320">{{cite journal| author=Bertario L, Russo A, Sala P, Eboli M, Giarola M, D'amico F et al.| title=Genotype and phenotype factors as determinants of desmoid tumors in patients with familial adenomatous polyposis. | journal=Int J Cancer | year= 2001 | volume= 95 | issue= 2 | pages= 102-7 | pmid=11241320 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11241320 }} </ref><ref name="pmid11247896">{{cite journal| author=Friedl W, Caspari R, Sengteller M, Uhlhaas S, Lamberti C, Jungck M et al.| title=Can APC mutation analysis contribute to therapeutic decisions in familial adenomatous polyposis? Experience from 680 FAP families. | journal=Gut | year= 2001 | volume= 48 | issue= 4 | pages= 515-21 | pmid=11247896 | doi= | pmc=1728231 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11247896 }} </ref> | *[[Familial]] [[desmoid tumor]] ([[germline]] [[APC]] [[mutation]]) occurs in 10% to 25% of [[FAP]] [[patients]]<ref name="pmid11241320">{{cite journal| author=Bertario L, Russo A, Sala P, Eboli M, Giarola M, D'amico F et al.| title=Genotype and phenotype factors as determinants of desmoid tumors in patients with familial adenomatous polyposis. | journal=Int J Cancer | year= 2001 | volume= 95 | issue= 2 | pages= 102-7 | pmid=11241320 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11241320 }} </ref><ref name="pmid11247896">{{cite journal| author=Friedl W, Caspari R, Sengteller M, Uhlhaas S, Lamberti C, Jungck M et al.| title=Can APC mutation analysis contribute to therapeutic decisions in familial adenomatous polyposis? Experience from 680 FAP families. | journal=Gut | year= 2001 | volume= 48 | issue= 4 | pages= 515-21 | pmid=11247896 | doi= | pmc=1728231 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11247896 }} </ref> | ||
*People with germline APC mutation have 850 times greater risk of developing desmoids than the general population<ref name="pmid16739877">{{cite journal| author=Ferenc T, Sygut J, Kopczyński J, Mayer M, Latos-Bieleńska A, Dziki A et al.| title=Aggressive fibromatosis (desmoid tumors): definition, occurrence, pathology, diagnostic problems, clinical behavior, genetic background. | journal=Pol J Pathol | year= 2006 | volume= 57 | issue= 1 | pages= 5-15 | pmid=16739877 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16739877 }} </ref> | *People with [[germline]] [[APC]] [[mutation]] have 850 times greater risk of developing [[Desmoid tumor|desmoids]] than the general [[population]]<ref name="pmid16739877">{{cite journal| author=Ferenc T, Sygut J, Kopczyński J, Mayer M, Latos-Bieleńska A, Dziki A et al.| title=Aggressive fibromatosis (desmoid tumors): definition, occurrence, pathology, diagnostic problems, clinical behavior, genetic background. | journal=Pol J Pathol | year= 2006 | volume= 57 | issue= 1 | pages= 5-15 | pmid=16739877 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16739877 }} </ref> | ||
*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<ref name="pmid21225148">{{cite journal| author=Leal RF, Silva PV, Ayrizono Mde L, Fagundes JJ, Amstalden EM, Coy CS| title=Desmoid tumor in patients with familial adenomatous polyposis. | journal=Arq Gastroenterol | year= 2010 | volume= 47 | issue= 4 | pages= 373-8 | pmid=21225148 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21225148 }} </ref><ref name="pmid24378386">{{cite journal| author=Fisher C, Thway K| title=Aggressive fibromatosis. | journal=Pathology | year= 2014 | volume= 46 | issue= 2 | pages= 135-40 | pmid=24378386 | doi=10.1097/PAT.0000000000000045 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24378386 }} </ref><ref name="pmid23334997">{{cite journal| author=Schiessling S, Kihm M, Ganschow P, Kadmon G, Büchler MW, Kadmon M| title=Desmoid tumour biology in patients with familial adenomatous polyposis coli. | journal=Br J Surg | year= 2013 | volume= 100 | issue= 5 | pages= 694-703 | pmid=23334997 | doi=10.1002/bjs.9053 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23334997 }} </ref><ref name="pmid16736290">{{cite journal| author=Fallen T, Wilson M, Morlan B, Lindor NM| title=Desmoid tumors -- a characterization of patients seen at Mayo Clinic 1976-1999. | journal=Fam Cancer | year= 2006 | volume= 5 | issue= 2 | pages= 191-4 | pmid=16736290 | doi=10.1007/s10689-005-5959-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16736290 }} </ref> | *Sporadic [[Desmoid tumor|desmoids]] ([[APC]] or the [[Beta-catenin|beta-catenin gene]] [[mutations]]) are relatively rare, affect 2 to 5 individuals per million per year in the general [[population]]<ref name="pmid21225148">{{cite journal| author=Leal RF, Silva PV, Ayrizono Mde L, Fagundes JJ, Amstalden EM, Coy CS| title=Desmoid tumor in patients with familial adenomatous polyposis. | journal=Arq Gastroenterol | year= 2010 | volume= 47 | issue= 4 | pages= 373-8 | pmid=21225148 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21225148 }} </ref><ref name="pmid24378386">{{cite journal| author=Fisher C, Thway K| title=Aggressive fibromatosis. | journal=Pathology | year= 2014 | volume= 46 | issue= 2 | pages= 135-40 | pmid=24378386 | doi=10.1097/PAT.0000000000000045 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24378386 }} </ref><ref name="pmid23334997">{{cite journal| author=Schiessling S, Kihm M, Ganschow P, Kadmon G, Büchler MW, Kadmon M| title=Desmoid tumour biology in patients with familial adenomatous polyposis coli. | journal=Br J Surg | year= 2013 | volume= 100 | issue= 5 | pages= 694-703 | pmid=23334997 | doi=10.1002/bjs.9053 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23334997 }} </ref><ref name="pmid16736290">{{cite journal| author=Fallen T, Wilson M, Morlan B, Lindor NM| title=Desmoid tumors -- a characterization of patients seen at Mayo Clinic 1976-1999. | journal=Fam Cancer | year= 2006 | volume= 5 | issue= 2 | pages= 191-4 | pmid=16736290 | doi=10.1007/s10689-005-5959-5 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16736290 }} </ref> | ||
===Incidence=== | ===Incidence=== | ||
*The incidence of desmoid tumor is approximately two to four per million population per year in general population<ref name="pmid7091046">{{cite journal| author=Reitamo JJ, Häyry P, Nykyri E, Saxén E| title=The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the Finnish population. | journal=Am J Clin Pathol | year= 1982 | volume= 77 | issue= 6 | pages= 665-73 | pmid=7091046 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7091046 }} </ref><ref name="aaa">Desmoid tumor. Dr Tim Luijkx and Radswiki et al.Radiopaedia 2015.http://radiopaedia.org/articles/desmoid-tumour. Accessed on January 21, 2016</ref> | *The [[incidence]] of [[desmoid tumor]] is approximately two to four per million [[population]] per year in general [[population]]<ref name="pmid7091046">{{cite journal| author=Reitamo JJ, Häyry P, Nykyri E, Saxén E| title=The desmoid tumor. I. Incidence, sex-, age- and anatomical distribution in the Finnish population. | journal=Am J Clin Pathol | year= 1982 | volume= 77 | issue= 6 | pages= 665-73 | pmid=7091046 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7091046 }} </ref><ref name="aaa">Desmoid tumor. Dr Tim Luijkx and Radswiki et al.Radiopaedia 2015.http://radiopaedia.org/articles/desmoid-tumour. Accessed on January 21, 2016</ref> | ||
===Gender=== | ===Gender=== | ||
* Women of fertile age (especially after childbirth) are more commonly affected with desmoid tumor than men<ref name="pmid19877160">{{cite journal| author=Mankin HJ, Hornicek FJ, Springfield DS| title=Extra-abdominal desmoid tumors: a report of 234 cases. | journal=J Surg Oncol | year= 2010 | volume= 102 | issue= 5 | pages= 380-4 | pmid=19877160 | doi=10.1002/jso.21433 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19877160 }} </ref> | * Women of fertile [[age]] (especially after [[childbirth]]) are more commonly affected with [[desmoid tumor]] than [[men]]<ref name="pmid19877160">{{cite journal| author=Mankin HJ, Hornicek FJ, Springfield DS| title=Extra-abdominal desmoid tumors: a report of 234 cases. | journal=J Surg Oncol | year= 2010 | volume= 102 | issue= 5 | pages= 380-4 | pmid=19877160 | doi=10.1002/jso.21433 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19877160 }} </ref> | ||
* Gender ratio is 2:1 for females to males | * Gender ratio is 2:1 for [[females]] to [[males]] | ||
* Incidence is the same in both genders in children | * [[Incidence]] is the same in both genders in [[children]] | ||
===Age=== | ===Age=== | ||
*Desmoid tumor commonly affects individuals between 20 to 60 years of age<ref name="aaa">Desmoid tumor. Dr Tim Luijkx and Radswiki et al.Radiopaedia 2015.http://radiopaedia.org/articles/desmoid-tumour. Accessed on January 21, 2016</ref><ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref> | *[[Desmoid tumor]] commonly affects individuals between 20 to 60 years of [[age]]<ref name="aaa">Desmoid tumor. Dr Tim Luijkx and Radswiki et al.Radiopaedia 2015.http://radiopaedia.org/articles/desmoid-tumour. Accessed on January 21, 2016</ref><ref name="EconomouPitta2011">{{cite journal|last1=Economou|first1=Athanasios|last2=Pitta|first2=Xanthi|last3=Andreadis|first3=Efstathios|last4=Papapavlou|first4=Leonidas|last5=Chrissidis|first5=Thomas|title=Desmoid tumor of the abdominal wall: a case report|journal=Journal of Medical Case Reports|volume=5|issue=1|year=2011|pages=326|issn=1752-1947|doi=10.1186/1752-1947-5-326}}</ref> | ||
*Average age is 30’s to 40’s | *Average [[age]] is 30’s to 40’s | ||
*Desmoids are rare in young and elderly | *[[Desmoid tumor|Desmoids]] are rare in young and [[elderly]] | ||
==Reference== | ==Reference== | ||
Revision as of 16:54, 13 March 2019
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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 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.