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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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:T2
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*heterogenous intermediate signal
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:T1 contrast + gadolinium
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*variable enhancement (moderate to intense)
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Primary (cutaneous origin)
A) intraepithelial cutaneous Paget disease of the usual type POS: CK7; GCDFP; CEA;
NEG: CK20; UPK
B) intraepithelial cutaneous Paget disease with invasion
C) intraepithelial cutaneous Paget disease as a manifestation of underlying
adenocarcinoma of skin appendage
Secondary (of non-cutaneous origin)
A) anorectal origin POS: CK20; CEA;
NEG: CK7; GCDFP; UPK
B) urothelial origin POS: CK7; UPK; CK20;
NEG: GCDFP15; CEA
C) other origin Antigen expression depends on primary tumor




Revision as of 17:04, 1 February 2016

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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 four subtypes based on site of origin and area affected.

Classification

The classification of extramammary Paget's disease 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]
Type of extramammary Paget's disease Features Useful markers
Primary (cutaneous origin)
  • 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

  • Positive markers: Cytokeratin (CK7); Gross cystic disease

fluid protein (GCDFP); Carcinoembryonic antigen (CEA)

  • Negative markers: Uroplakin (UPK); Cytokeratin (CK20)
T2
  • heterogenous intermediate signal
T1 contrast + gadolinium
  • variable enhancement (moderate to intense)


Primary (cutaneous origin) A) intraepithelial cutaneous Paget disease of the usual type POS: CK7; GCDFP; CEA; NEG: CK20; UPK B) intraepithelial cutaneous Paget disease with invasion C) intraepithelial cutaneous Paget disease as a manifestation of underlying adenocarcinoma of skin appendage Secondary (of non-cutaneous origin) A) anorectal origin POS: CK20; CEA; NEG: CK7; GCDFP; UPK B) urothelial origin POS: CK7; UPK; CK20; NEG: GCDFP15; CEA C) 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.


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