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

Desmoid tumor Microchapters

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Overview

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Pathophysiology

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Differentiating Desmoid tumor from other Diseases

Epidemiology and Demographics

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Case #1

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

Prevalence

Incidence

Gender

Age

Reference

  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. Fisher C, Thway K (2014). "Aggressive fibromatosis". Pathology. 46 (2): 135–40. doi:10.1097/PAT.0000000000000045. PMID 24378386.
  6. 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.
  7. 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.
  8. 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. 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
  10. 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.
  11. 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.

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