Extramammary Paget's disease epidemiology and demographics: Difference between revisions

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==Overview==
==Overview==
Extramammary Paget's disease is rare and the precise incidence is unknown. Extramammary Paget's disease commonly affects individuals 50-60 years of age. Females are more commonly affected with the disease than males. The female to male ratio is approximately 4.5-3 to 1. It usually affects individuals of the [[Caucasian]] race, but it may occur in other races.<ref name="pmid3001158">{{cite journal| author=Chanda JJ| title=Extramammary Paget's disease: prognosis and relationship to internal malignancy. | journal=J Am Acad Dermatol | year= 1985 | volume= 13 | issue= 6 | pages= 1009-14 | pmid=3001158 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3001158  }} </ref>
Extramammary Paget's disease is rare, and its exact incidence is unknown. Extramammary Paget's disease commonly affects individuals 50-60 years of age. Females are more commonly affected with the disease than males. The female to male ratio is approximately 3-4.5 to 1. It usually affects individuals of the [[Caucasian]] race, but it may occur in other races.<ref name="pmid3001158">{{cite journal| author=Chanda JJ| title=Extramammary Paget's disease: prognosis and relationship to internal malignancy. | journal=J Am Acad Dermatol | year= 1985 | volume= 13 | issue= 6 | pages= 1009-14 | pmid=3001158 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3001158  }} </ref>
==Epidemiology and Demographics==
==Epidemiology and Demographics==
*Isolated [[Paget's disease]] of the penis is rare and only a few cases have been reported in the literature. In men, the penoscrotal region is the most commonly affected area.<ref name="pmid15882734">{{cite journal| author=Yang WJ, Kim DS, Im YJ, Cho KS, Rha KH, Cho NH et al.| title=Extramammary Paget's disease of penis and scrotum. | journal=Urology | year= 2005 | volume= 65 | issue= 5 | pages= 972-5 | pmid=15882734 | doi=10.1016/j.urology.2004.12.010 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15882734  }} </ref>
*The vulva remains the most frequently involved site with approximately 65% of extramammary Paget’s disease located in this area.<ref name="AsmoukiOumouloud2012">{{cite journal|last1=Asmouki|first1=Hamid|last2=Oumouloud|first2=Rachid|last3=Aboulfalah|first3=Abderrahim|last4=Soummani|first4=Abderraouf|last5=Marrat|first5=Abdelouahed|title=Paget’s Disease of the Vulva in Premenopausal Woman Treated with Only Surgery: A Case Report|journal=Case Reports in Oncological Medicine|volume=2012|year=2012|pages=1–4|issn=2090-6706|doi=10.1155/2012/854827}}</ref>
*Vulval extramammary Paget's disease represents 1% to 5% of all vulval malignancies, with a peak age incidence of 65 years.<ref name="pmid2175288">{{cite journal| author=Curtin JP, Rubin SC, Jones WB, Hoskins WJ, Lewis JL| title=Paget's disease of the vulva. | journal=Gynecol Oncol | year= 1990 | volume= 39 | issue= 3 | pages= 374-7 | pmid=2175288 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2175288  }} </ref><ref name="pmid11064666">{{cite journal| author=Lloyd J, Flanagan AM| title=Mammary and extramammary Paget's disease. | journal=J Clin Pathol | year= 2000 | volume= 53 | issue= 10 | pages= 742-9 | pmid=11064666 | doi= | pmc=PMC1731095 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11064666  }} </ref>
*Extramammary Paget disease can also occur in association with invasive carcinomas of the [[genitourinary]] or [[gastrointestinal]] tract. The frequency of that association varies from 9% to 32%. Invasive genitourinary tract cancers or gastrointestinal sites can be associated, synchronously or metachronously, with [[penoscrotal]] extramammary Paget's disease, but the frequency of this association has not been reported.
*The majority of extramammary Paget's disease findings (approximately 76%) are accounted by vulvar EMPD, although vulvar EMPD remains a rare neoplastic finding, representing less than 1% of the vulvar neoplasms. Between 17% and 30% of the patients with EMPD may have an underlying [[adenocarcinoma]].
*A higher rate of underlying adenocarcinoma was found in patients with perianal involvement and palpable masses in the vulvar region. In approximately 10-20% of cases EMPD is associated to coexisting malignancies at other sites, as the [[breast]], the rectum,the skin basal cells, the genitourinary tract and the cervix.
*A relationship between the site of EMPD and the probability to find an adenocarcinoma of cutaneous adnexal structures or an internal malignancy, has been suggested by a Spanish study. The study reports that vulvar EMPD was associated with adnexal adenocarcinoma in 4% of cases and with a distant malignancy in 20%, while perianal EMPD was associated with adnexal adenocarcinoma in 7% of cases and with an internal malignancy in 14%.<ref name="MorettoNair2013">{{cite journal|last1=Moretto|first1=P.|last2=Nair|first2=V.J.|last3=El Hallani|first3=S.|last4=Malone|first4=S.|last5=Belanger|first5=E.|last6=Morash|first6=C.|last7=Canil|first7=C.M.|title=Management of penoscrotal extramammary Paget disease: case series and review of the literature|journal=Current Oncology|volume=20|issue=4|year=2013|pages=311|issn=1718-7729|doi=10.3747/co.20.1353}}</ref>
===Incidence===
===Incidence===
Extramammary Paget's disease is rare and the precise incidence is unknown. However, in older reports, it represented 6.5% of all cases of Paget disease. According to a recent European study the incidence of invasive extramammary paget's disease is approximately 0.6 per 100,000 individuals worldwide.<ref name="MorettoNair2013">{{cite journal|last1=Moretto|first1=P.|last2=Nair|first2=V.J.|last3=El Hallani|first3=S.|last4=Malone|first4=S.|last5=Belanger|first5=E.|last6=Morash|first6=C.|last7=Canil|first7=C.M.|title=Management of penoscrotal extramammary Paget disease: case series and review of the literature|journal=Current Oncology|volume=20|issue=4|year=2013|pages=311|issn=1718-7729|doi=10.3747/co.20.1353}}</ref>
* Extramammary Paget's disease is rare, and the exact incidence is unknown.
===Prevalence===
* The incidence of extramammary Paget's disease is estimated to be 0.10 per 100,000 individuals.
The prevalence of invasive extramammary Paget's disease is estimated to occur in 5-25% of patients.  
* However, in older reports, it represented 6.5% of all cases of all Paget diseases.
 
===Age===
===Age===
Extramammary Paget's disease commonly affects individuals 50-60 years of age. The median age of incidence of vulval disease was 65 years. The median age for a diagnosis of scrotal disease was 70.4 years; for penile disease, it was 73.6 years. After exclusion of vulvar disease, extramammary Paget's disease was most commonly seen in the scrotum and penis, followed by the anorectal area.<ref name="MorettoNair2013">{{cite journal|last1=Moretto|first1=P.|last2=Nair|first2=V.J.|last3=El Hallani|first3=S.|last4=Malone|first4=S.|last5=Belanger|first5=E.|last6=Morash|first6=C.|last7=Canil|first7=C.M.|title=Management of penoscrotal extramammary Paget disease: case series and review of the literature|journal=Current Oncology|volume=20|issue=4|year=2013|pages=311|issn=1718-7729|doi=10.3747/co.20.1353}}</ref>
* The peak age incidence of vulvar extramammary Paget's disease is approximately 50-65 years.<ref name="pmid2175288">{{cite journal| author=Curtin JP, Rubin SC, Jones WB, Hoskins WJ, Lewis JL| title=Paget's disease of the vulva. | journal=Gynecol Oncol | year= 1990 | volume= 39 | issue= 3 | pages= 374-7 | pmid=2175288 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2175288  }} </ref><ref name="pmid11064666">{{cite journal| author=Lloyd J, Flanagan AM| title=Mammary and extramammary Paget's disease. | journal=J Clin Pathol | year= 2000 | volume= 53 | issue= 10 | pages= 742-9 | pmid=11064666 | doi= | pmc=PMC1731095 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11064666  }} </ref>
The median age at diagnosis for extramammary Paget's disease is found to be 72 years. <ref name="GuerraMisra2013">{{cite journal|last1=Guerra|first1=Rosa|last2=Misra|first2=Subhasis|title=Management of Extramammary Paget's Disease: A Case Report and Review of the Literature|journal=Case Reports in Dermatological Medicine|volume=2013|year=2013|pages=1–4|issn=2090-6463|doi=10.1155/2013/436390}}</ref> The median age at diagnosis for extramammary Paget's disease is found to be 72 years.<ref name="GuerraMisra2013">{{cite journal|last1=Guerra|first1=Rosa|last2=Misra|first2=Subhasis|title=Management of Extramammary Paget's Disease: A Case Report and Review of the Literature|journal=Case Reports in Dermatological Medicine|volume=2013|year=2013|pages=1–4|issn=2090-6463|doi=10.1155/2013/436390}}</ref>
* The median age for a diagnosis of scrotal and penile disease is approximately 70-75 years.
 
===Gender===
===Gender===
Females are more commonly affected with extramammary Paget's disease than males. The female to male ratio is approximately 4.5-3 to 1.<ref name="pmid3001158">{{cite journal| author=Chanda JJ| title=Extramammary Paget's disease: prognosis and relationship to internal malignancy. | journal=J Am Acad Dermatol | year= 1985 | volume= 13 | issue= 6 | pages= 1009-14 | pmid=3001158 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3001158  }} </ref>
Females are more commonly affected with extramammary Paget's disease than males. The female to male ratio is approximately 3-4.5 to 1.<ref name="pmid3001158">{{cite journal| author=Chanda JJ| title=Extramammary Paget's disease: prognosis and relationship to internal malignancy. | journal=J Am Acad Dermatol | year= 1985 | volume= 13 | issue= 6 | pages= 1009-14 | pmid=3001158 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3001158  }} </ref>
===Race===
===Race===
Extramammary Paget's disease usually affects individuals of the Caucasian race, but it may occur in other races.<ref name="ZolloZeitouni2000">{{cite journal|last1=Zollo|first1=J.D.|last2=Zeitouni|first2=N.C.|title=The Roswell Park Cancer Institute experience with extramammary Paget’s disease|journal=British Journal of Dermatology|volume=142|issue=1|year=2000|pages=59–65|issn=00070963|doi=10.1046/j.1365-2133.2000.03242.x}}</ref>
Extramammary Paget's disease usually affects individuals of the Caucasian race, but it may occur in other races.<ref name="ZolloZeitouni2000">{{cite journal|last1=Zollo|first1=J.D.|last2=Zeitouni|first2=N.C.|title=The Roswell Park Cancer Institute experience with extramammary Paget’s disease|journal=British Journal of Dermatology|volume=142|issue=1|year=2000|pages=59–65|issn=00070963|doi=10.1046/j.1365-2133.2000.03242.x}}</ref>

Revision as of 20:13, 5 February 2016

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]

Overview

Extramammary Paget's disease is rare, and its exact incidence is unknown. Extramammary Paget's disease commonly affects individuals 50-60 years of age. Females are more commonly affected with the disease than males. The female to male ratio is approximately 3-4.5 to 1. It usually affects individuals of the Caucasian race, but it may occur in other races.[1]

Epidemiology and Demographics

Incidence

  • Extramammary Paget's disease is rare, and the exact incidence is unknown.
  • The incidence of extramammary Paget's disease is estimated to be 0.10 per 100,000 individuals.
  • However, in older reports, it represented 6.5% of all cases of all Paget diseases.

Age

  • The peak age incidence of vulvar extramammary Paget's disease is approximately 50-65 years.[2][3]
  • The median age for a diagnosis of scrotal and penile disease is approximately 70-75 years.

Gender

Females are more commonly affected with extramammary Paget's disease than males. The female to male ratio is approximately 3-4.5 to 1.[1]

Race

Extramammary Paget's disease usually affects individuals of the Caucasian race, but it may occur in other races.[4]

References

  1. ↑ 1.0 1.1 Chanda JJ (1985). "Extramammary Paget's disease: prognosis and relationship to internal malignancy". J Am Acad Dermatol. 13 (6): 1009–14. PMID 3001158.
  2. ↑ Curtin JP, Rubin SC, Jones WB, Hoskins WJ, Lewis JL (1990). "Paget's disease of the vulva". Gynecol Oncol. 39 (3): 374–7. PMID 2175288.
  3. ↑ Lloyd J, Flanagan AM (2000). "Mammary and extramammary Paget's disease". J Clin Pathol. 53 (10): 742–9. PMC 1731095. PMID 11064666.
  4. ↑ Zollo, J.D.; Zeitouni, N.C. (2000). "The Roswell Park Cancer Institute experience with extramammary Paget's disease". British Journal of Dermatology. 142 (1): 59–65. doi:10.1046/j.1365-2133.2000.03242.x. ISSN 0007-0963.


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