Extramammary Paget's disease epidemiology and demographics
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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.