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 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> | ||
==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> | *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> | *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> | *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. | *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. | *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 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> | *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 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> | ||
Revision as of 19:01, 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 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.[1]
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.[2]
- The vulva remains the most frequently involved site with approximately 65% of extramammary Paget’s disease located in this area.[3]
- Vulval extramammary Paget's disease represents 1% to 5% of all vulval malignancies, with a peak age incidence of 65 years.[4][5]
- 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%.[6]
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.[6]
Prevalence
The prevalence of invasive extramammary Paget's disease is estimated to occur in 5-25% of patients.
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.[6] The median age at diagnosis for extramammary Paget's disease is found to be 72 years. [7] The median age at diagnosis for extramammary Paget's disease is found to be 72 years.[7]
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.[1]
Race
Extramammary Paget's disease usually affects individuals of the Caucasian race, but it may occur in other races.[8]
References
- ↑ 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.
- ↑ Yang WJ, Kim DS, Im YJ, Cho KS, Rha KH, Cho NH; et al. (2005). "Extramammary Paget's disease of penis and scrotum". Urology. 65 (5): 972–5. doi:10.1016/j.urology.2004.12.010. PMID 15882734.
- ↑ Asmouki, Hamid; Oumouloud, Rachid; Aboulfalah, Abderrahim; Soummani, Abderraouf; Marrat, Abdelouahed (2012). "Paget's Disease of the Vulva in Premenopausal Woman Treated with Only Surgery: A Case Report". Case Reports in Oncological Medicine. 2012: 1–4. doi:10.1155/2012/854827. ISSN 2090-6706.
- ↑ 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.
- ↑ Lloyd J, Flanagan AM (2000). "Mammary and extramammary Paget's disease". J Clin Pathol. 53 (10): 742–9. PMC 1731095. PMID 11064666.
- ↑ 6.0 6.1 6.2 Moretto, P.; Nair, V.J.; El Hallani, S.; Malone, S.; Belanger, E.; Morash, C.; Canil, C.M. (2013). "Management of penoscrotal extramammary Paget disease: case series and review of the literature". Current Oncology. 20 (4): 311. doi:10.3747/co.20.1353. ISSN 1718-7729.
- ↑ 7.0 7.1 Guerra, Rosa; Misra, Subhasis (2013). "Management of Extramammary Paget's Disease: A Case Report and Review of the Literature". Case Reports in Dermatological Medicine. 2013: 1–4. doi:10.1155/2013/436390. ISSN 2090-6463.
- ↑ 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.