Extramammary Paget's disease classification: Difference between revisions

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==Overview==
==Overview==
Extramammary Paget's disease may be classified into four subtypes based on site of origin and area affected.
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.
==Classification==
==Classification==
===Classification Based on Affected Area===
The classification of extramammary Paget's disease based on the site and area affeced is shown below in a tabular form:


The classification of extramammary Paget's disease is shown below in a tabular form:
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! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Type of Extramammary Paget's Disease}}
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! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Description}}
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:Vulval Extramammary Paget's Disease
:Vulval Extramammary Paget's Disease
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*The majority of cases of vulval extramammary Paget's disease are primary; that is, arising within the epidermis, and very few are associated with cutaneous sweat gland tumors.  
*The majority of cases of vulval extramammary Paget's disease are primary; that is, arising within the epidermis, and very few are associated with cutaneous [[sweat gland]] [[tumors]].  
*In cases where there is a prominent dermal invasive component, it may be impossible to prove the primary site of origin of the tumor.
*In cases where there is a prominent dermal invasive component, it may be impossible to prove the primary site of origin of the tumor.
*A new variant of “mammary like” cutaneous glands, which combines morphological features of eccrine, mammary, and apocrine glands, and occurs predominantly in the interlabial sulcus on the vulva, has also been proposed as the site of origin of sweat gland derived tumors from which extramammary Paget's disease arises.  
*A new variant of “mammary like” cutaneous glands, which combines morphological features of eccrine, mammary, and apocrine glands, and occurs predominantly in the interlabial sulcus on the vulva, has also been proposed as the site of origin of sweat gland derived tumors from which extramammary Paget's disease arises.  
*Vulval extramammary Paget's disease has been described in association with endocervical, endometrial, vaginal, vulval (eg., Bartholin's gland), urethral, and bladder neoplasms.
*Vulval extramammary Paget's disease has been described in association with endocervical, [[endometrial]], vaginal, vulval (eg., [[Bartholin's gland]]), [[urethral]], and [[bladder]] neoplasms.
*This phenomenon of epidermal colonisation by cells from a neoplasm originating within local internal organs with epithelial linings contiguous with the affected skin is described in many articles as spread from “internal malignancy”.
*This phenomenon of epidermal colonisation by cells from a neoplasm originating within local internal organs with epithelial linings contiguous with the affected skin is described in many articles as spread from “internal malignancy”.
*Rare reports exist of extramammary Paget's disease associated with distant tumors arising in organs without a direct epithelial connection to the affected epidermis (ovarian carcinoma, hepatocellular carcinoma, bile duct carcinoma, and renal cell carcinoma).  
*Rare reports exist of extramammary Paget's disease associated with distant tumors arising in organs without a direct epithelial connection to the affected [[epidermis]] ([[ovarian carcinoma]], [[hepatocellular carcinoma]], [[bile duct carcinoma]], and [[renal cell carcinoma]]).  
*Vulval extramammary Paget's disease has also been described in association with breast carcinoma. It is likely that such cases simply represent synchronous coincidental neoplasms. No evidence exists in any of the described cases that the Paget's cells originated from the internal malignancy.<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="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="pmid9914572">{{cite journal| author=Fanning J, Lambert HC, Hale TM, Morris PC, Schuerch C| title=Paget's disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision. | journal=Am J Obstet Gynecol | year= 1999 | volume= 180 | issue= 1 Pt 1 | pages= 24-7 | pmid=9914572 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9914572  }} </ref><ref name="pmid1666822">{{cite journal| author=van der Putte SC| title=Anogenital "sweat" glands. Histology and pathology of a gland that may mimic mammary glands. | journal=Am J Dermatopathol | year= 1991 | volume= 13 | issue= 6 | pages= 557-67 | pmid=1666822 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1666822  }} </ref><ref name="pmid8040464">{{cite journal| author=van der Putte SC, van Gorp LH| title=Adenocarcinoma of the mammary-like glands of the vulva: a concept unifying sweat gland carcinoma of the vulva, carcinoma of supernumerary mammary glands and extramammary Paget's disease. | journal=J Cutan Pathol | year= 1994 | volume= 21 | issue= 2 | pages= 157-63 | pmid=8040464 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8040464  }} </ref><ref name="pmid2993379">{{cite journal| author=Powell FC, Bjornsson J, Doyle JA, Cooper AJ| title=Genital Paget's disease and urinary tract malignancy. | journal=J Am Acad Dermatol | year= 1985 | volume= 13 | issue= 1 | pages= 84-90 | pmid=2993379 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2993379  }} </ref><ref name="pmid9155118">{{cite journal| author=Koga F, Gotoh S, Suzuki S| title=[A case of invasive bladder cancer with Pagetoid skin lesion of the vulva and anogenital Paget's disease]. | journal=Nihon Hinyokika Gakkai Zasshi | year= 1997 | volume= 88 | issue= 4 | pages= 503-6 | pmid=9155118 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9155118  }} </ref><ref name="pmid2837997">{{cite journal| author=Hayashibara Y, Ikeda S| title=[Extramammary Paget's disease with internal malignancies]. | journal=Gan To Kagaku Ryoho | year= 1988 | volume= 15 | issue= 4 Pt 2-3 | pages= 1569-75 | pmid=2837997 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2837997  }} </ref><ref name="pmid7872301">{{cite journal| author=Nakano S, Narita R, Tabaru A, Ogami Y, Otsuki M| title=Bile duct cancer associated with extramammary Paget's disease. | journal=Am J Gastroenterol | year= 1995 | volume= 90 | issue= 3 | pages= 507-8 | pmid=7872301 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7872301  }} </ref><ref name="pmid9784335">{{cite journal| author=Popiolek DA, Hajdu SI, Gal D| title=Synchronous Paget's disease of the vulva and breast. | journal=Gynecol Oncol | year= 1998 | volume= 71 | issue= 1 | pages= 137-40 | pmid=9784335 | doi=10.1006/gyno.1998.5136 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9784335  }} </ref>
*Vulval extramammary Paget's disease has also been described in association with [[breast carcinoma]]. It is likely that such cases simply represent synchronous coincidental neoplasms. No evidence exists in any of the described cases that the Paget's cells originated from the internal malignancy.<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="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="pmid9914572">{{cite journal| author=Fanning J, Lambert HC, Hale TM, Morris PC, Schuerch C| title=Paget's disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision. | journal=Am J Obstet Gynecol | year= 1999 | volume= 180 | issue= 1 Pt 1 | pages= 24-7 | pmid=9914572 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9914572  }} </ref><ref name="pmid1666822">{{cite journal| author=van der Putte SC| title=Anogenital "sweat" glands. Histology and pathology of a gland that may mimic mammary glands. | journal=Am J Dermatopathol | year= 1991 | volume= 13 | issue= 6 | pages= 557-67 | pmid=1666822 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1666822  }} </ref><ref name="pmid8040464">{{cite journal| author=van der Putte SC, van Gorp LH| title=Adenocarcinoma of the mammary-like glands of the vulva: a concept unifying sweat gland carcinoma of the vulva, carcinoma of supernumerary mammary glands and extramammary Paget's disease. | journal=J Cutan Pathol | year= 1994 | volume= 21 | issue= 2 | pages= 157-63 | pmid=8040464 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8040464  }} </ref><ref name="pmid2993379">{{cite journal| author=Powell FC, Bjornsson J, Doyle JA, Cooper AJ| title=Genital Paget's disease and urinary tract malignancy. | journal=J Am Acad Dermatol | year= 1985 | volume= 13 | issue= 1 | pages= 84-90 | pmid=2993379 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2993379  }} </ref><ref name="pmid9155118">{{cite journal| author=Koga F, Gotoh S, Suzuki S| title=[A case of invasive bladder cancer with Pagetoid skin lesion of the vulva and anogenital Paget's disease]. | journal=Nihon Hinyokika Gakkai Zasshi | year= 1997 | volume= 88 | issue= 4 | pages= 503-6 | pmid=9155118 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9155118  }} </ref><ref name="pmid2837997">{{cite journal| author=Hayashibara Y, Ikeda S| title=[Extramammary Paget's disease with internal malignancies]. | journal=Gan To Kagaku Ryoho | year= 1988 | volume= 15 | issue= 4 Pt 2-3 | pages= 1569-75 | pmid=2837997 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2837997  }} </ref><ref name="pmid7872301">{{cite journal| author=Nakano S, Narita R, Tabaru A, Ogami Y, Otsuki M| title=Bile duct cancer associated with extramammary Paget's disease. | journal=Am J Gastroenterol | year= 1995 | volume= 90 | issue= 3 | pages= 507-8 | pmid=7872301 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7872301  }} </ref><ref name="pmid9784335">{{cite journal| author=Popiolek DA, Hajdu SI, Gal D| title=Synchronous Paget's disease of the vulva and breast. | journal=Gynecol Oncol | year= 1998 | volume= 71 | issue= 1 | pages= 137-40 | pmid=9784335 | doi=10.1006/gyno.1998.5136 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9784335  }} </ref>
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:Axillary Extramammary Paget's Disease
:Axillary Extramammary Paget's Disease
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*Axillary extramammary Paget's disease may, arise within the epidermis or from neoplasms in the local apocrine glands, however exclusion of neoplastic disease in the breast tissue is mandatory before a diagnosis of primary extramammary Paget's disease is made in this site.  
*Axillary extramammary Paget's disease may, arise within the [[epidermis]] or from neoplasms in the local apocrine glands, however exclusion of neoplastic disease in the breast tissue is mandatory before a diagnosis of primary extramammary Paget's disease is made in this site.  
*Analysis of c-erbB-2 expression may be of use in this differential diagnosis.
*Analysis of c-erbB-2 expression may be of use in this differential diagnosis.
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:Perianal Extramammary Paget's Disease
:Perianal Extramammary Paget's Disease
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*Perianal extramammary Paget's disease is rare than vulval disease but is strongly associated with adenocarcinoma of the colorectum and anus.
*Perianal extramammary Paget's disease is rare than vulval disease but is strongly associated with [[adenocarcinoma]] of the colorectum and anus.
*Unlike vulval extramammary Paget's disease, approximately 70–80% of cases of perianal disease arise secondary to invasive malignancy in the rectum, anus, or colon.
*Unlike vulval extramammary Paget's disease, approximately 70–80% of cases of perianal disease arise secondary to invasive malignancy in the [[rectum]], anus, or colon.
*Thorough investigation of perianal region is mandatory if a diagnosis of perianal Paget's disease is given.<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>
*Thorough investigation of perianal region is mandatory if a diagnosis of perianal Paget's disease is given.<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>
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:Paget's Disease of Male Genitalia
:Paget's Disease of male genitalia
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*Disease of the male genitalia is thought to be more frequently associated with internal malignancy (eg., bladder, urethral, prostatic, and testicular neoplasms) than is vulval extramammary Paget's disease.<ref name="pmid2993379">{{cite journal| author=Powell FC, Bjornsson J, Doyle JA, Cooper AJ| title=Genital Paget's disease and urinary tract malignancy. | journal=J Am Acad Dermatol | year= 1985 | volume= 13 | issue= 1 | pages= 84-90 | pmid=2993379 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2993379  }} </ref><ref name="pmid9155118">{{cite journal| author=Koga F, Gotoh S, Suzuki S| title=[A case of invasive bladder cancer with Pagetoid skin lesion of the vulva and anogenital Paget's disease]. | journal=Nihon Hinyokika Gakkai Zasshi | year= 1997 | volume= 88 | issue= 4 | pages= 503-6 | pmid=9155118 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9155118  }} </ref><ref name="pmid9640381">{{cite journal| author=Allan SJ, McLaren K, Aldridge RD| title=Paget's disease of the scrotum: a case exhibiting positive prostate-specific antigen staining and associated prostatic adenocarcinoma. | journal=Br J Dermatol | year= 1998 | volume= 138 | issue= 4 | pages= 689-91 | pmid=9640381 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9640381  }} </ref>
*Disease of the male genitalia is thought to be more frequently associated with internal malignancy (eg., bladder, urethral, prostatic, and testicular neoplasms) than is vulval extramammary Paget's disease.<ref name="pmid2993379">{{cite journal| author=Powell FC, Bjornsson J, Doyle JA, Cooper AJ| title=Genital Paget's disease and urinary tract malignancy. | journal=J Am Acad Dermatol | year= 1985 | volume= 13 | issue= 1 | pages= 84-90 | pmid=2993379 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2993379  }} </ref><ref name="pmid9155118">{{cite journal| author=Koga F, Gotoh S, Suzuki S| title=[A case of invasive bladder cancer with Pagetoid skin lesion of the vulva and anogenital Paget's disease]. | journal=Nihon Hinyokika Gakkai Zasshi | year= 1997 | volume= 88 | issue= 4 | pages= 503-6 | pmid=9155118 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9155118  }} </ref><ref name="pmid9640381">{{cite journal| author=Allan SJ, McLaren K, Aldridge RD| title=Paget's disease of the scrotum: a case exhibiting positive prostate-specific antigen staining and associated prostatic adenocarcinoma. | journal=Br J Dermatol | year= 1998 | volume= 138 | issue= 4 | pages= 689-91 | pmid=9640381 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9640381  }} </ref>
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===Classification Based on Origin of Paget's Cells===
* Extramammary Paget's disease may be classified based on the origin of the Paget's cells according to a [[Classification|classification system]] proposed by Wilkinson and Brown into two groups: primary ([[cutaneous]]) and secondary (non-[[cutaneous]]).


{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align=center
* Classification of extramammary Paget's disease into two groups is shown below in a tabular form:<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>
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! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Type of extramammary Paget's disease}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Features}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Useful markers}}
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:Primary (cutaneous origin)
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*Intraepithelial cutaneous Paget disease of the usual type
*Intraepithelial cutaneous Paget disease with invasion
*Intraepithelial cutaneous Paget disease as a manifestation of underlying adenocarcinoma of skin appendage
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*Positive markers: Cytokeratin (CK7); Gross cystic disease fluid protein (GCDFP);  Carcinoembryonic antigen (CEA)
*Negative markers: Uroplakin (UPK); Cytokeratin (CK20)
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:Secondary (of non-cutaneous origin)
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*Anorectal origin
*Urothelial origin
*Other origin
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*Positive markers: Cytokeratin (CK20); Carcinoembryonic antigen (CEA), Negative markers: Cytokeratin (CK7); Gross cystic disease fluid protein (GCDFP); Uroplakin
*Positive markers: Cytokeratin (CK7); Uroplakin (UPK); Cytokeratin (CK20), Negative markers:Gross cystic disease fluid protein (GCDFP15); Carcinoembryonic antigen (CEA)
*Other origin Antigen expression depends on primary tumor
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! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF| Type of extramammary Paget's disease}}
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF| Type of extramammary Paget's disease}}
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*Intraepithelial cutaneous Paget's disease of the usual type
*Intraepithelial cutaneous Paget's disease of the usual type
*Intraepithelial cutaneous Paget's disease with invasion
*Intraepithelial cutaneous Paget's disease with invasion
*Intraepithelial cutaneous Paget's disease as a manifestation of  underlying adenocracinoma of skin appendage
*Intraepithelial cutaneous Paget's disease as a manifestation of  underlying [[adenocarcinoma]] of skin appendage
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*Positive markers: Cytokeratin (CK7); Gross cystic disease fluid protein (GCDFP); Carcinoembryonic antigen (CEA)
*Positive markers: Cytokeratin (CK7); Gross cystic disease fluid protein (GCDFP); [[Carcinoembryonic antigen]] (CEA)
*Negative markers: Uroplakin (UPK); Cytokeratin (CK20)
*Negative markers: Uroplakin (UPK); Cytokeratin (CK20)
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| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" rowspan="3" | Secondary (non-cutaneous origin)
| rowspan="3" style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" | Secondary (non-cutaneous origin)
| style="padding: 5px 5px; background: #F5F5F5;" | Anorectal origin
| style="padding: 5px 5px; background: #F5F5F5;" | Anorectal origin
|style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*Positive markers: Cytokeratin (CK20); Carcinoembryonic antigen (CEA)
*Positive markers: Cytokeratin (CK20); Carcinoembryonic antigen (CEA)
*Negative markers: Cytokeratin (CK7); Gross cystic disease fluid protein (GCDFP): Uroplakin (UPK)
*Negative markers: Cytokeratin (CK7); Gross cystic disease fluid protein (GCDFP): Uroplakin(UPK)
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|style="padding: 5px 5px; background: #F5F5F5;" | Urothelial origin
| style="padding: 5px 5px; background: #F5F5F5;" | Urothelial origin
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*Positive markers: Cytokeratin (CK7); Cytokeratin (CK20); Uroplakin (UPK)
*Positive markers: Cytokeratin (CK7); Cytokeratin (CK20); Uroplakin (UPK)
*Negative markers: Gross cystic disease fluid protein (GCDFP15); Carcinoembryonic antigen (CEA)
*Negative markers: Gross cystic disease fluid protein (GCDFP15); [[Carcinoembryonic antigen]] (CEA)
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|style="padding: 5px 5px; background: #F5F5F5;" | Other origin
| style="padding: 5px 5px; background: #F5F5F5;" | Other origin
|style="padding: 5px 5px; background: #F5F5F5;" | Other origin antigen expression depends on primary tumor
| style="padding: 5px 5px; background: #F5F5F5;" | Other origin antigen expression depends on primary tumor
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==References==
==References==
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Latest revision as of 14:04, 9 April 2019

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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 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.

Classification

Classification Based on Affected Area

The classification of extramammary Paget's disease based on the site and area affeced is shown below in a tabular form:

Type of Extramammary Paget's Disease Description
Vulval Extramammary Paget's Disease
  • The majority of cases of vulval extramammary Paget's disease are primary; that is, arising within the epidermis, and very few are associated with cutaneous sweat gland tumors.
  • In cases where there is a prominent dermal invasive component, it may be impossible to prove the primary site of origin of the tumor.
  • A new variant of “mammary like” cutaneous glands, which combines morphological features of eccrine, mammary, and apocrine glands, and occurs predominantly in the interlabial sulcus on the vulva, has also been proposed as the site of origin of sweat gland derived tumors from which extramammary Paget's disease arises.
  • Vulval extramammary Paget's disease has been described in association with endocervical, endometrial, vaginal, vulval (eg., Bartholin's gland), urethral, and bladder neoplasms.
  • This phenomenon of epidermal colonisation by cells from a neoplasm originating within local internal organs with epithelial linings contiguous with the affected skin is described in many articles as spread from “internal malignancy”.
  • Rare reports exist of extramammary Paget's disease associated with distant tumors arising in organs without a direct epithelial connection to the affected epidermis (ovarian carcinoma, hepatocellular carcinoma, bile duct carcinoma, and renal cell carcinoma).
  • Vulval extramammary Paget's disease has also been described in association with breast carcinoma. It is likely that such cases simply represent synchronous coincidental neoplasms. No evidence exists in any of the described cases that the Paget's cells originated from the internal malignancy.[1][2][3][4][5][6][7][8][9][10]
Axillary Extramammary Paget's Disease
  • Axillary extramammary Paget's disease may, arise within the epidermis or from neoplasms in the local apocrine glands, however exclusion of neoplastic disease in the breast tissue is mandatory before a diagnosis of primary extramammary Paget's disease is made in this site.
  • Analysis of c-erbB-2 expression may be of use in this differential diagnosis.
Perianal Extramammary Paget's Disease
  • Perianal extramammary Paget's disease is rare than vulval disease but is strongly associated with adenocarcinoma of the colorectum and anus.
  • Unlike vulval extramammary Paget's disease, approximately 70–80% of cases of perianal disease arise secondary to invasive malignancy in the rectum, anus, or colon.
  • Thorough investigation of perianal region is mandatory if a diagnosis of perianal Paget's disease is given.[11]
Paget's Disease of male genitalia
  • Disease of the male genitalia is thought to be more frequently associated with internal malignancy (eg., bladder, urethral, prostatic, and testicular neoplasms) than is vulval extramammary Paget's disease.[6][7][12]

Classification Based on Origin of Paget's Cells

  • Extramammary Paget's disease may be classified based on the origin of the Paget's cells according to a classification system proposed by Wilkinson and Brown into two groups: primary (cutaneous) and secondary (non-cutaneous).
  • Classification of extramammary Paget's disease into two groups is shown below in a tabular form:[13]
Type of extramammary Paget's disease Features Useful marker
Primary (cutaneous origin)
  • Intraepithelial cutaneous Paget's disease of the usual type
  • Intraepithelial cutaneous Paget's disease with invasion
  • Intraepithelial cutaneous Paget's disease as a manifestation of underlying adenocarcinoma of skin appendage
  • Positive markers: Cytokeratin (CK7); Gross cystic disease fluid protein (GCDFP); Carcinoembryonic antigen (CEA)
  • Negative markers: Uroplakin (UPK); Cytokeratin (CK20)
Secondary (non-cutaneous origin) Anorectal origin
  • Positive markers: Cytokeratin (CK20); Carcinoembryonic antigen (CEA)
  • Negative markers: Cytokeratin (CK7); Gross cystic disease fluid protein (GCDFP): Uroplakin(UPK)
Urothelial origin
  • Positive markers: Cytokeratin (CK7); Cytokeratin (CK20); Uroplakin (UPK)
  • Negative markers: Gross cystic disease fluid protein (GCDFP15); Carcinoembryonic antigen (CEA)
Other origin Other origin antigen expression depends on primary tumor

References

  1. ↑ 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.
  2. ↑ Goldblum JR, Hart WR (1997). "Vulvar Paget's disease: a clinicopathologic and immunohistochemical study of 19 cases". Am J Surg Pathol. 21 (10): 1178–87. PMID 9331290.
  3. ↑ Fanning J, Lambert HC, Hale TM, Morris PC, Schuerch C (1999). "Paget's disease of the vulva: prevalence of associated vulvar adenocarcinoma, invasive Paget's disease, and recurrence after surgical excision". Am J Obstet Gynecol. 180 (1 Pt 1): 24–7. PMID 9914572.
  4. ↑ van der Putte SC (1991). "Anogenital "sweat" glands. Histology and pathology of a gland that may mimic mammary glands". Am J Dermatopathol. 13 (6): 557–67. PMID 1666822.
  5. ↑ van der Putte SC, van Gorp LH (1994). "Adenocarcinoma of the mammary-like glands of the vulva: a concept unifying sweat gland carcinoma of the vulva, carcinoma of supernumerary mammary glands and extramammary Paget's disease". J Cutan Pathol. 21 (2): 157–63. PMID 8040464.
  6. ↑ 6.0 6.1 Powell FC, Bjornsson J, Doyle JA, Cooper AJ (1985). "Genital Paget's disease and urinary tract malignancy". J Am Acad Dermatol. 13 (1): 84–90. PMID 2993379.
  7. ↑ 7.0 7.1 Koga F, Gotoh S, Suzuki S (1997). "[A case of invasive bladder cancer with Pagetoid skin lesion of the vulva and anogenital Paget's disease]". Nihon Hinyokika Gakkai Zasshi. 88 (4): 503–6. PMID 9155118.
  8. ↑ Hayashibara Y, Ikeda S (1988). "[Extramammary Paget's disease with internal malignancies]". Gan To Kagaku Ryoho. 15 (4 Pt 2-3): 1569–75. PMID 2837997.
  9. ↑ Nakano S, Narita R, Tabaru A, Ogami Y, Otsuki M (1995). "Bile duct cancer associated with extramammary Paget's disease". Am J Gastroenterol. 90 (3): 507–8. PMID 7872301.
  10. ↑ Popiolek DA, Hajdu SI, Gal D (1998). "Synchronous Paget's disease of the vulva and breast". Gynecol Oncol. 71 (1): 137–40. doi:10.1006/gyno.1998.5136. PMID 9784335.
  11. ↑ Goldblum JR, Hart WR (1998). "Perianal Paget's disease: a histologic and immunohistochemical study of 11 cases with and without associated rectal adenocarcinoma". Am J Surg Pathol. 22 (2): 170–9. PMID 9500217.
  12. ↑ Allan SJ, McLaren K, Aldridge RD (1998). "Paget's disease of the scrotum: a case exhibiting positive prostate-specific antigen staining and associated prostatic adenocarcinoma". Br J Dermatol. 138 (4): 689–91. PMID 9640381.
  13. ↑ Wilkinson EJ, Brown HM (2002). "Vulvar Paget disease of urothelial origin: a report of three cases and a proposed classification of vulvar Paget disease". Hum Pathol. 33 (5): 549–54. PMID 12094382.


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