Extramammary Paget's disease classification: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
No edit summary
No edit summary
 
(6 intermediate revisions by 3 users not shown)
Line 3: Line 3:
{{CMG}}{{AE}}{{Simrat}}
{{CMG}}{{AE}}{{Simrat}}
==Overview==
==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.<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.
==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 based on the site and area affeced is shown below in a tabular form:
{| style="border: 0px; font-size: 90%; margin: 3px; width: 80%" align="center"
{| style="border: 0px; font-size: 90%; margin: 3px; width: 700px" align=center
| valign="top" |
|valign=top|
|+
|+
! style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Type of Extramammary Paget's Disease}}
! style="background: #4479BA; width: 20%;" | {{fontcolor|#FFF|Type of Extramammary Paget's Disease}}
! style="background: #4479BA; width: 400px;" | {{fontcolor|#FFF|Description}}
! style="background: #4479BA; width: 60%;" | {{fontcolor|#FFF|Description}}
|-
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" |
Line 19: Line 20:
*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]]).  
Line 44: Line 45:
|}
|}


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 Based on Origin of Paget's Cells===
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>
* 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
 
|valign=top|
* 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>
{| style="border: 0px; font-size: 90%; margin: 3px; width: 600px" align="center"
| valign="top" |
|+
|+
! 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}}
Line 62: Line 65:
*Negative markers: Uroplakin (UPK); Cytokeratin (CK20)
*Negative markers: Uroplakin (UPK); Cytokeratin (CK20)
|-
|-
| 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)
|-
|-
|style="padding: 5px 5px; background: #F5F5F5;" | Urothelial origin
| style="padding: 5px 5px; background: #F5F5F5;" | Urothelial origin
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*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)
|-
|-
|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
|}
|}


Line 87: Line 90:
{{WikiDoc Help Menu}}
{{WikiDoc Help Menu}}
{{WikiDoc Sources}}
{{WikiDoc Sources}}
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Dermatology]]
[[Category:Surgery]]

Latest revision as of 14:04, 9 April 2019

Extramammary Paget's disease Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Extramammary Paget's disease from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Chest X Ray

CT

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Extramammary Paget's disease classification On the Web

Most recent articles

cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Extramammary Paget's disease classification

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Extramammary Paget's disease classification

CDC on Extramammary Paget's disease classification

Extramammary Paget's disease classification in the news

Blogs on Extramammary Paget's disease classification

Directions to Hospitals Treating Extramammary Paget's disease

Risk calculators and risk factors for Extramammary Paget's disease classification

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.


Template:WikiDoc Sources

Retrieved from "https://www.wikidoc.org/index.php?title=Extramammary_Paget%27s_disease_classification&oldid=1562151"