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| ==Overview== | | ==Overview== |
| Extamammary Paget's disease (EMPD) is a rare cutaneous, intraepithelial adenocarcinoma involving primarily the epidermis but occasionally extending into the underlying dermis. It has predilection for apocrine gland-bearing areas: mostly the perineum, vulva, axilla, scrotum and penis. The origin of the neoplastic cells could be [[apocrine gland]]s or epithelial [[stem cells]]. Extramammary paget's disease may originate in vulvar apocrine-gland bearing skin cells or as a manifestation of adjacent primary rectal, anal, or bladder adenocarcinoma. The most common site of involvement is the vulva, although perianal, perineal, scrotal and penile skin may also be affected. Extramammary Paget disease (empd) is also described as an apocrine gland tumour occurring in both a benign and a malignant form with metastatic potential. It can present as carcinoma in situ or as invasive disease that can subsequently metastasize to lymph nodes and distant sites.<ref name="MannLavaf2012">{{cite journal|last1=Mann|first1=J.|last2=Lavaf|first2=A.|last3=Tejwani|first3=A.|last4=Ross|first4=P.|last5=Ashamalla|first5=H.|title=Perianal Paget disease treated definitively with radiotherapy|journal=Current Oncology|volume=19|issue=6|year=2012|issn=11980052|doi=10.3747/co.19.1144}}</ref><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><ref name="ShepherdDavidson2005">{{cite journal|last1=Shepherd|first1=Victoria|last2=Davidson|first2=Emma J.|last3=Davies-Humphreys|first3=John|title=Extramammary Paget's disease|journal=BJOG: An International Journal of Obstetrics and Gynaecology|volume=112|issue=3|year=2005|pages=273–279|issn=1470-0328|doi=10.1111/j.1471-0528.2004.00438.x}}</ref><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><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> Extramammary Paget's disease was first discovered by Radcliffe Crocker, in 1889.<ref name="wiki"> extramammary Paget's disease. Wikipedia(2015) https://en.wikipedia.org/wiki/Extramammary_Paget%27s_disease Accessed on January 26, 2016</ref> Extramammary Paget's disease may be classified into two groups based on the origin of the Paget's cells. Extramammary Paget's disease may be classified into four subtypes based on site of origin and area affected.<ref name="pmid12094382">{{cite journal| author=Wilkinson EJ, Brown HM| title=Vulvar Paget disease of urothelial origin: a report of three cases and a proposed classification of vulvar Paget disease. | journal=Hum Pathol | year= 2002 | volume= 33 | issue= 5 | pages= 549-54 | pmid=12094382 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12094382 }} </ref> On gross pathology, plaque with an irregular border and erythematous or white lesion are characteristic findings of extramammary Paget's disease. On microscopic histopathological analysis, Paget's cells which are large cells with abundant amphophilic or basophilic, finely granular cytoplasm, the nucleus which is usually large, centrally situated, and sometimes contains a prominent nucleolus, and signet ring cells are characteristic findings of extramammary Paget's disease. Extramammary Paget's disease arises from keratinocytic stem cells or from apocrine gland ducts. Approximately 25% (range, 9-32%) of the cases of EMPD are associated with an underlying in situ or invasive neoplasm. The neoplasm most likely to be associated with EMPD is an adnexal apocrine carcinoma. This associated neoplasm probably represents infiltration of the deeper adnexa by epidermal Paget cells.<ref name="pmid19888952">{{cite journal| author=Roy J, Mirnezami A, Gatt M, Sasapu KK, Scott N, Sagar PM| title=A rare case of Paget's disease in a retrorectal dermoid cyst. | journal=Colorectal Dis | year= 2010 | volume= 12 | issue= 9 | pages= 946-7 | pmid=19888952 | doi=10.1111/j.1463-1318.2009.02102.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19888952 }} </ref><ref name="librepathology"> Ameloblastoma. Libre pathology(2015) http://librepathology.org/wiki/index.php/Extramammary_Paget_disease#Microscopic Accessed on January 30, 2016</ref> The cause of extramammary Paget's disease has not been identified.<ref>Lever, Walter F., and David E. Elder. Lever's histopathology of the skin. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2009.</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><ref>Mehregan, Amir H., and Hermann Pinkus. Pinkus' guide to dermatohistopathology. Norwalk, Conn: Appleton-Century-Crofts, 1986. Print.</ref><ref name="OrlandiPiccione2001">{{cite journal|last1=Orlandi|first1=A.|last2=Piccione|first2=E.|last3=Francesconi|first3=A.|last4=Spagnoli|first4=L. G.|title=Simultaneous vulvar intraepithelial neoplasia and Paget's disease: Report of two cases|journal=International Journal of Gynecological Cancer|volume=11|issue=3|year=2001|pages=224–228|issn=1048-891X|doi=10.1046/j.1525-1438.2001.01016.x}}</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> Symptoms of extramammary Paget's disease include pruritis, vulvar pain, vulvar bleeding, and burning sensation.<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><ref name="pmid10739709">{{cite journal| author=Parker LP, Parker JR, Bodurka-Bevers D, Deavers M, Bevers MW, Shen-Gunther J et al.| title=Paget's disease of the vulva: pathology, pattern of involvement, and prognosis. | journal=Gynecol Oncol | year= 2000 | volume= 77 | issue= 1 | pages= 183-9 | pmid=10739709 | doi=10.1006/gyno.2000.5741 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10739709 }} </ref><ref name="pmid12193912">{{cite journal| author=Tebes S, Cardosi R, Hoffman M| title=Paget's disease of the vulva. | journal=Am J Obstet Gynecol | year= 2002 | volume= 187 | issue= 2 | pages= 281-3; discussion 283-4 | pmid=12193912 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12193912 }} </ref> Biopsy is diagnostic of extramammary Paget's disease. Other diagnostic studies for extramammary Paget's disease include fine needle aspirate and PAP smear. Other imaging studies for EMPD include bone scan, ultrasound scan, PET scan, cystoscopy, sigmoidoscopy, colonoscopy, mammography, and colposcopy, which demonstrates metastases and underlying invasive carcinomas.<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><ref name="pmid15692486">{{cite journal| author=Cho SB, Yun M, Lee MG, Chung KY| title=Variable patterns of positron emission tomography in the assessment of patients with extramammary Paget's disease. | journal=J Am Acad Dermatol | year= 2005 | volume= 52 | issue= 2 | pages= 353-5 | pmid=15692486 | doi=10.1016/j.jaad.2004.10.864 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15692486 }} </ref> | | Extamammary Paget's disease (EMPD) is a rare non-melanocytic [[Epidermal|intraepidermal]] [[skin lesion]]. It usually involves the [[epidermis]], but occasionally extends into the underlying [[dermis]]. It has predilection for [[apocrine gland]]-bearing areas: mostly the [[perineum]], [[vulva]], [[axilla]], [[scrotum]] and [[penis]]. Extramammary Paget's disease may be classified into two groups based on the origin of the [[Paget's disease|Paget's cells]]. Extramammary Paget's disease may be classified into four subtypes based on site of origin and area affected. On [[gross pathology]], [[plaque]] with an irregular border and [[erythematous]] or white [[lesion]] are characteristic findings of extramammary Paget's disease. On microscopic [[Histopathological|histopathological analysis]], the presence of Paget's cells (large cells with abundant amphophilic or [[basophilic]], finely granular [[cytoplasm]] and a large, centrally-located [[nucleus]] and prominent [[nucleolus]]) and [[Signet ring cell|signet ring cells]] are characteristic findings of extramammary Paget's disease. Extramammary Paget's disease is usually associated with [[adnexal]] [[apocrine]] [[carcinoma]], which represents infiltration of the deeper [[adnexa]] by [[epidermal]] Paget cells. Neither the direct cause nor a prominent risk factor of extramammary Paget's disease has been identified. Extramammary Paget's disease is rare and the exact [[incidence]] is unknown. Extramammary Paget's disease commonly affects individuals 50-60 years of age. [[Female|Females]] are more commonly affected with the disease than [[Male|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. Symptoms of extramammary Paget's disease include [[pruritis]], [[Vulva|vulvar]] pain, [[Vulva|vulvar]] [[bleeding]], and burning sensation. [[Biopsy]] is diagnostic of extramammary Paget's disease. Other diagnostic studies for extramammary Paget's disease include [[Fine needle aspiration|fine needle aspirate]] and [[Pap smear|PAP smear]]. [[Treatment Planning|Treatment]] depends on the stage at [[diagnosis]]. Treatment often includes [[surgery]] and [[chemotherapy]] with either [[5-fluorouracil]], [[imiquimod]], or combination of [[paclitaxel]] and [[trastuzumab]]. |
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| ==Historical Perspective== | | ==Historical Perspective== |
| Extramammary Paget's disease was first discovered by Radcliffe Crocker, in 1889.<ref name="wiki"> extramammary Paget's disease. Wikipedia(2015) https://en.wikipedia.org/wiki/Extramammary_Paget%27s_disease Accessed on January 26, 2016</ref > | | Extramammary Paget's disease was first discovered by Radcliffe Crocker in 1889. |
| ==Classification== | | ==Classification== |
| Extramammary Paget's disease may be classified into two groups based on the origin of the Paget's cells. Extramammary Paget's disease may be classified into four subtypes based on site of origin and area affected.<ref name="pmid12094382">{{cite journal| author=Wilkinson EJ, Brown HM| title=Vulvar Paget disease of urothelial origin: a report of three cases and a proposed classification of vulvar Paget disease. | journal=Hum Pathol | year= 2002 | volume= 33 | issue= 5 | pages= 549-54 | pmid=12094382 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12094382 }} </ref> | | Extramammary Paget's disease may be classified into two groups based on the origin of the Paget's cells. Extramammary Paget's disease may be classified into four subtypes based on site of origin and area affected. |
| ==Pathophysiology== | | ==Pathophysiology== |
| On gross pathology, plaque with an irregular border and erythematous or white lesion are characteristic findings of extramammary Paget's disease. On microscopic histopathological analysis, Paget's cells which are large cells with abundant amphophilic or basophilic, finely granular cytoplasm, the nucleus which is usually large, centrally situated, and sometimes contains a prominent nucleolus, and signet ring cells are characteristic findings of extramammary Paget's disease. Extramammary Paget's disease arises from keratinocytic stem cells or from apocrine gland ducts. Approximately 25% (range, 9-32%) of the cases of EMPD are associated with an underlying in situ or invasive neoplasm. The neoplasm most likely to be associated with EMPD is an adnexal apocrine carcinoma. This associated neoplasm probably represents infiltration of the deeper adnexa by epidermal Paget cells.<ref name="pmid19888952">{{cite journal| author=Roy J, Mirnezami A, Gatt M, Sasapu KK, Scott N, Sagar PM| title=A rare case of Paget's disease in a retrorectal dermoid cyst. | journal=Colorectal Dis | year= 2010 | volume= 12 | issue= 9 | pages= 946-7 | pmid=19888952 | doi=10.1111/j.1463-1318.2009.02102.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19888952 }} </ref><ref name="librepathology"> Ameloblastoma. Libre pathology(2015) http://librepathology.org/wiki/index.php/Extramammary_Paget_disease#Microscopic Accessed on January 30, 2016</ref> | | On [[gross pathology]], [[plaque]] with an irregular border and [[erythematous]] or white [[lesion]] are characteristic findings of extramammary Paget's disease. On microscopic [[Histopathological|histopathological analysis]], [[Paget's cells]] which are large cells with abundant amphophilic or [[basophilic]], finely granular [[cytoplasm]], the [[nucleus]] which is usually large, centrally situated, and sometimes contains a prominent [[nucleolus]], and signet ring cells are characteristic findings of extramammary Paget's disease. Extramammary Paget's disease arises from keratinocytic [[stem cells]] or from [[apocrine gland]] ducts. Approximately 25% (range 9-32%) of the cases of extramammary Paget's disease are associated with an underlying in situ or invasive [[neoplasm]]. Extramammary Paget's disease is usually associated with [[adnexal]] [[apocrine]] [[carcinoma]], which represents infiltration of the deeper [[adnexa]] by [[epidermal]] [[Paget's disease|Paget cells]]. |
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| ==Causes== | | ==Causes== |
| The cause of extramammary Paget's disease has not been identified.<ref>Lever, Walter F., and David E. Elder. Lever's histopathology of the skin. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2009.</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><ref>Mehregan, Amir H., and Hermann Pinkus. Pinkus' guide to dermatohistopathology. Norwalk, Conn: Appleton-Century-Crofts, 1986. Print.</ref><ref name="OrlandiPiccione2001">{{cite journal|last1=Orlandi|first1=A.|last2=Piccione|first2=E.|last3=Francesconi|first3=A.|last4=Spagnoli|first4=L. G.|title=Simultaneous vulvar intraepithelial neoplasia and Paget's disease: Report of two cases|journal=International Journal of Gynecological Cancer|volume=11|issue=3|year=2001|pages=224–228|issn=1048-891X|doi=10.1046/j.1525-1438.2001.01016.x}}</ref> | | The cause of extramammary Paget's disease has not been identified. |
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| ==Differential Diagnosis== | | ==Differential Diagnosis== |
| Extramammary Paget's disease must be differentiated from basal cell carcinoma, Bowen disease, cutaneous candidiasis, intertrigo, irritant contact dermatitis, lichen simplex chronicus, plaque psoriasis, tinea cruris, seborrhoeic dermatitis, lichen sclerosis, anogenital intraepithelial neoplasia, melanoma, histiocytosis, mycosis fungoides, leucoplakia, squamous cell carcinoma, condylomata accuminata, Crohn's disease, and hidradenitis suppurativa.<ref name="pmid2844363">{{cite journal| author=Balducci L, Crawford ED, Smith GF, Lambuth B, McGehee R, Hardy C| title=Extramammary Paget's disease: an annotated review. | journal=Cancer Invest | year= 1988 | volume= 6 | issue= 3 | pages= 293-303 | pmid=2844363 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2844363 }} </ref> | | Extramammary Paget's disease must be differentiated from [[basal cell carcinoma]], [[Bowen's disease]], cutaneous [[candidiasis]], [[intertrigo]], [[irritant contact dermatitis]], [[lichen simplex chronicus]], plaque [[psoriasis]], [[tinea cruris]], [[seborrhoeic dermatitis]], lichen sclerosis, anogenital intraepithelial neoplasia, [[melanoma]], [[histiocytosis]], [[mycosis fungoides]], [[leukoplakia]], [[squamous cell carcinoma]], condylomata accuminata, [[Crohn's disease]], and [[hidradenitis suppurativa]]. |
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| ==Epidemiology and Demographics== | | ==Epidemiology and Demographics== |
| 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. [[Female|Females]] are more commonly affected with the disease than [[Male|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. |
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| ==Risk Factors== | | ==Risk Factors== |
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| ==Screening== | | ==Screening== |
| According to the United States Preventive Services Task Force, screening for extramammary Paget's disease is not recommended.<ref name=screening>http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=extramammary+pagets+disease Accessed on January 26, 2016.</ref> | | According to the United States Preventive Services Task Force, screening for extramammary Paget's disease is not recommended among the general population. |
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| ==Natural History, Complications and Prognosis== | | ==Natural History, Complications and Prognosis== |
| If left untreated, the disease always takes a progressive course with no spontaneous recovery. Common complications of extramammary Paget's disease include recurrence of the tumor. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. The prognosis for primary Extramammary Pagets's disease (EMPD) confined to the epidermis is excellent. However, invasive primary extramammary Paget's disease (EMPD) carries a poor prognosis, particularly if lymphovascular invasion is present.<ref name="pmid9331290">{{cite journal| author=Goldblum JR, Hart WR| title=Vulvar Paget's disease: a clinicopathologic and immunohistochemical study of 19 cases. | journal=Am J Surg Pathol | year= 1997 | volume= 21 | issue= 10 | pages= 1178-87 | pmid=9331290 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9331290 }} </ref><ref name="pmid9500217">{{cite journal| author=Goldblum JR, Hart WR| title=Perianal Paget's disease: a histologic and immunohistochemical study of 11 cases with and without associated rectal adenocarcinoma. | journal=Am J Surg Pathol | year= 1998 | volume= 22 | issue= 2 | pages= 170-9 | pmid=9500217 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9500217 }} </ref><ref>DeVita, Vincent T., Theodore S. Lawrence, and Steven A. Rosenberg. DeVita, Hellman, and Rosenberg's cancer : principles & practice of oncology. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins, 2011. Print.</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><ref name="MannLavaf2012">{{cite journal|last1=Mann|first1=J.|last2=Lavaf|first2=A.|last3=Tejwani|first3=A.|last4=Ross|first4=P.|last5=Ashamalla|first5=H.|title=Perianal Paget disease treated definitively with radiotherapy|journal=Current Oncology|volume=19|issue=6|year=2012|issn=11980052|doi=10.3747/co.19.1144}}</ref><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> | | If left untreated, the disease is usually progressive. Common complications of extramammary Paget's disease include [[recurrence]] of the [[tumor]] and [[metastasis]]. Depending on the extent of the [[tumor]] at the time of diagnosis, the [[prognosis]] may vary. The [[prognosis]] for primary extramammary Pagets's disease confined to the [[Epidermis (skin)|epidermis]] is excellent. However, invasive primary extramammary Paget's disease carries a poor [[prognosis]], particularly if lymphovascular invasion is present. |
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| | ==Diagnosis== |
| | ===History and Symptoms=== |
| | Symptoms of extramammary Paget's disease include [[pruritis]], [[Vulva|vulvar]] pain, [[Vulva|vulvar]] bleeding, and burning sensation. |
| | ===Physical Examination=== |
| | Common physical examination findings of extramammary Paget's disease include well demarcated, [[erythematous]] or leucoplakic [[Plaque|plaques]] present on the [[skin]], characteristic ‘cake-icing’ appearance of [[Vulva|vulval]] extramammary Paget's disease ([[Erythema|erythematous]] changes associated with white islands and bridges of hyperkeratotic [[epithelium]]), and [[lymphadenopathy]]. |
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| | ===Chest X Ray=== |
| | [[X-rays|Chest x-rays]] may be performed to detect [[Metastasis|metastases]] of extramammary Paget's disease to the [[Lung|lungs]]. |
| | ===CT=== |
| | [[Chest]], [[abdomen]], and [[Pelvis|pelvic]] [[Computed tomography|CT scan]] may be helpful in the diagnosis of extramammary Paget's disease. [[Computed tomography|CT scan]] can confirm the exact location of the [[cancer]] and show the [[Organ (anatomy)|organs]] nearby, as well as [[lymph nodes]] and distant [[Organ (anatomy)|organs]] where the [[cancer]] might have spread. |
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| ==History and Symptoms== | | ===MRI=== |
| Symptoms of extramammary Paget's disease include pruritis, vulvar pain, vulvar bleeding, and burning sensation.<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><ref name="pmid10739709">{{cite journal| author=Parker LP, Parker JR, Bodurka-Bevers D, Deavers M, Bevers MW, Shen-Gunther J et al.| title=Paget's disease of the vulva: pathology, pattern of involvement, and prognosis. | journal=Gynecol Oncol | year= 2000 | volume= 77 | issue= 1 | pages= 183-9 | pmid=10739709 | doi=10.1006/gyno.2000.5741 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10739709 }} </ref><ref name="pmid12193912">{{cite journal| author=Tebes S, Cardosi R, Hoffman M| title=Paget's disease of the vulva. | journal=Am J Obstet Gynecol | year= 2002 | volume= 187 | issue= 2 | pages= 281-3; discussion 283-4 | pmid=12193912 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12193912 }} </ref>
| | [[Chest]], [[abdomen]], and [[Pelvis|pelvic]] [[Magnetic resonance imaging|MRI scan]] may be helpful in the diagnosis of EMPD. |
| ==Physical Examination==
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| Common physical examination findings of extramammary Paget's disease include well demarcated, erythematous or leucoplakic plaques present on the skin, characteristic ‘cake-icing’ appearance of vulval extramammary Paget's disease, consisting of erythematous changes associated with white islands and bridges of hyperkeratotic epithelium, and lymphadenopathy.<ref name="pmid8393362">{{cite journal| author=Heymann WR| title=Extramammary Paget's disease. | journal=Clin Dermatol | year= 1993 | volume= 11 | issue= 1 | pages= 83-7 | pmid=8393362 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8393362 }} </ref><ref>Silverberg, Steven G., and Ronald A. DeLellis. Silverberg's principles and practice of surgical pathology and cytopathology. Edinburgh: Churchill Livingstone/Elsevier, 2006. Print.</ref>
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| ==Chest X Ray== | | ===Other Imaging Findings=== |
| Chest x-rays may be performed to detect metastases of extramammary Paget's disease to the lungs.
| | Other imaging studies for EMPD include [[bone scan]], [[ultrasound scan]], [[PET scan]], [[cystoscopy]], [[sigmoidoscopy]], [[colonoscopy]], [[mammography]], and [[colposcopy]], which demonstrates [[metastases]] and underlying [[Carcinoma|invasive carcinomas]]. |
| ==CT==
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| Chest, abdomen, and pelvic CT scan may be helpful in the diagnosis of extramammary Paget's disease. CT scan can confirm the exact location of the cancer and show the organs nearby, as well as lymph nodes and distant organs where the cancer might have spread.
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| ==MRI== | | ===Other Diagnostic Studies=== |
| Chest, abdomen, and pelvic MRI scan may be helpful in the diagnosis of EMPD.
| | Biopsy is diagnostic of extramammary Paget's disease. Other diagnostic studies for extramammary Paget's disease include [[Fine needle aspiration|fine needle aspirate]] and [[Pap smear]]. |
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| ==Other Imaging Findings== | | ==Treatment== |
| Other imaging studies for EMPD include bone scan, ultrasound scan, PET scan, cystoscopy, sigmoidoscopy, colonoscopy, mammography, and colposcopy, which demonstrates metastases and underlying invasive carcinomas.<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><ref name="pmid15692486">{{cite journal| author=Cho SB, Yun M, Lee MG, Chung KY| title=Variable patterns of positron emission tomography in the assessment of patients with extramammary Paget's disease. | journal=J Am Acad Dermatol | year= 2005 | volume= 52 | issue= 2 | pages= 353-5 | pmid=15692486 | doi=10.1016/j.jaad.2004.10.864 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15692486 }} </ref>
| | ===Medical Therapy=== |
| | Treatment depends on the stage at diagnosis. [[Chemotherapy]] with either [[5-fluorouracil]], [[imiquimod]], or combination of [[paclitaxel]] and [[trastuzumab]] has been evaluated for the treatment of extramammary Paget's disease. |
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| ==Other Diagnostic Studies== | | ===Surgery=== |
| Biopsy is diagnostic of extramammary Paget's disease. Other diagnostic studies for extramammary Paget's disease include fine needle aspirate and PAP smear.
| | [[Surgery]] is the first line treatment for extramammary Paget's disease. |
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| ==Medical Therapy== | | ===Primary Prevention=== |
| ==Surgery== | | There are no [[Primary prevention|primary preventive]] measures against extramammary Paget's disease. |
| ==Primary Prevention==
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| Biopsy is diagnostic of extramammary Paget's disease. Other diagnostic studies for extramammary Paget's disease include fine needle aspirate and PAP smear.
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| ==Secondary Prevention== | | ===Secondary Prevention=== |
| Secondary prevention strategies following extramammary Paget's disease include an annual complete physical examination, proctosigmoidoscopy and punch biopsy of any new lesion. Colonoscopy should be carried out at every two to three year intervals.<ref name="pmid9336114 [">{{cite journal| author=Sarmiento JM, Wolff BG, Burgart LJ, Frizelle FA, Ilstrup DM| title=Paget's disease of the perianal region--an aggressive disease? | journal=Dis Colon Rectum | year= 1997 | volume= 40 | issue= 10 | pages= 1187-94 | pmid=9336114 [ | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9336114 }} </ref> | | [[Secondary prevention]] strategies following extramammary Paget's disease include an annual complete physical examination, [[proctosigmoidoscopy]] and [[punch biopsy]] of any new lesion. [[Colonoscopy]] should be carried out at every two to three year intervals. |
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| ==References== | | ==References== |
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| | [[Category:Surgery]] |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Simrat Sarai, M.D. [2]
Overview
Extamammary Paget's disease (EMPD) is a rare non-melanocytic intraepidermal skin lesion. It usually involves the epidermis, but occasionally extends into the underlying dermis. It has predilection for apocrine gland-bearing areas: mostly the perineum, vulva, axilla, scrotum and penis. Extramammary Paget's disease may be classified into two groups based on the origin of the Paget's cells. Extramammary Paget's disease may be classified into four subtypes based on site of origin and area affected. On gross pathology, plaque with an irregular border and erythematous or white lesion are characteristic findings of extramammary Paget's disease. On microscopic histopathological analysis, the presence of Paget's cells (large cells with abundant amphophilic or basophilic, finely granular cytoplasm and a large, centrally-located nucleus and prominent nucleolus) and signet ring cells are characteristic findings of extramammary Paget's disease. Extramammary Paget's disease is usually associated with adnexal apocrine carcinoma, which represents infiltration of the deeper adnexa by epidermal Paget cells. Neither the direct cause nor a prominent risk factor of extramammary Paget's disease has been identified. Extramammary Paget's disease is rare and the 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. Symptoms of extramammary Paget's disease include pruritis, vulvar pain, vulvar bleeding, and burning sensation. Biopsy is diagnostic of extramammary Paget's disease. Other diagnostic studies for extramammary Paget's disease include fine needle aspirate and PAP smear. Treatment depends on the stage at diagnosis. Treatment often includes surgery and chemotherapy with either 5-fluorouracil, imiquimod, or combination of paclitaxel and trastuzumab.
Historical Perspective
Extramammary Paget's disease was first discovered by Radcliffe Crocker in 1889.
Classification
Extramammary Paget's disease may be classified into two groups based on the origin of the Paget's cells. Extramammary Paget's disease may be classified into four subtypes based on site of origin and area affected.
Pathophysiology
On gross pathology, plaque with an irregular border and erythematous or white lesion are characteristic findings of extramammary Paget's disease. On microscopic histopathological analysis, Paget's cells which are large cells with abundant amphophilic or basophilic, finely granular cytoplasm, the nucleus which is usually large, centrally situated, and sometimes contains a prominent nucleolus, and signet ring cells are characteristic findings of extramammary Paget's disease. Extramammary Paget's disease arises from keratinocytic stem cells or from apocrine gland ducts. Approximately 25% (range 9-32%) of the cases of extramammary Paget's disease are associated with an underlying in situ or invasive neoplasm. Extramammary Paget's disease is usually associated with adnexal apocrine carcinoma, which represents infiltration of the deeper adnexa by epidermal Paget cells.
Causes
The cause of extramammary Paget's disease has not been identified.
Differential Diagnosis
Extramammary Paget's disease must be differentiated from basal cell carcinoma, Bowen's disease, cutaneous candidiasis, intertrigo, irritant contact dermatitis, lichen simplex chronicus, plaque psoriasis, tinea cruris, seborrhoeic dermatitis, lichen sclerosis, anogenital intraepithelial neoplasia, melanoma, histiocytosis, mycosis fungoides, leukoplakia, squamous cell carcinoma, condylomata accuminata, Crohn's disease, and hidradenitis suppurativa.
Epidemiology and Demographics
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.
Risk Factors
There are no established risk factors for extramammary Paget's disease.
Screening
According to the United States Preventive Services Task Force, screening for extramammary Paget's disease is not recommended among the general population.
Natural History, Complications and Prognosis
If left untreated, the disease is usually progressive. Common complications of extramammary Paget's disease include recurrence of the tumor and metastasis. Depending on the extent of the tumor at the time of diagnosis, the prognosis may vary. The prognosis for primary extramammary Pagets's disease confined to the epidermis is excellent. However, invasive primary extramammary Paget's disease carries a poor prognosis, particularly if lymphovascular invasion is present.
Diagnosis
History and Symptoms
Symptoms of extramammary Paget's disease include pruritis, vulvar pain, vulvar bleeding, and burning sensation.
Physical Examination
Common physical examination findings of extramammary Paget's disease include well demarcated, erythematous or leucoplakic plaques present on the skin, characteristic ‘cake-icing’ appearance of vulval extramammary Paget's disease (erythematous changes associated with white islands and bridges of hyperkeratotic epithelium), and lymphadenopathy.
Chest X Ray
Chest x-rays may be performed to detect metastases of extramammary Paget's disease to the lungs.
CT
Chest, abdomen, and pelvic CT scan may be helpful in the diagnosis of extramammary Paget's disease. CT scan can confirm the exact location of the cancer and show the organs nearby, as well as lymph nodes and distant organs where the cancer might have spread.
MRI
Chest, abdomen, and pelvic MRI scan may be helpful in the diagnosis of EMPD.
Other Imaging Findings
Other imaging studies for EMPD include bone scan, ultrasound scan, PET scan, cystoscopy, sigmoidoscopy, colonoscopy, mammography, and colposcopy, which demonstrates metastases and underlying invasive carcinomas.
Other Diagnostic Studies
Biopsy is diagnostic of extramammary Paget's disease. Other diagnostic studies for extramammary Paget's disease include fine needle aspirate and Pap smear.
Treatment
Medical Therapy
Treatment depends on the stage at diagnosis. Chemotherapy with either 5-fluorouracil, imiquimod, or combination of paclitaxel and trastuzumab has been evaluated for the treatment of extramammary Paget's disease.
Surgery
Surgery is the first line treatment for extramammary Paget's disease.
Primary Prevention
There are no primary preventive measures against extramammary Paget's disease.
Secondary Prevention
Secondary prevention strategies following extramammary Paget's disease include an annual complete physical examination, proctosigmoidoscopy and punch biopsy of any new lesion. Colonoscopy should be carried out at every two to three year intervals.
References
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