Extramammary Paget's disease epidemiology and demographics: Difference between revisions
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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== | ||
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. 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. About that, a Spanish study suggests a relationship between the site of EMPD and the probability to find an adenocarcinoma of cutaneous adnexal structures or an internal malignancy. 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. | Extramammary Paget's disease is rare and the precise incidence is unknown. | ||
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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
Vulval extramammary Paget's disease represents 1% to 5% of all vulval malignancies, with a peak age incidence of 65 years.[2][3] 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. About that, a Spanish study suggests a relationship between the site of EMPD and the probability to find an adenocarcinoma of cutaneous adnexal structures or an internal malignancy. 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%.[4]
Incidence
Extramammary Paget's disease is rare and the precise incidence is unknown.
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.[4]
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.[5]
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.
- ↑ 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.
- ↑ 4.0 4.1 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.
- ↑ 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.