Extramammary Paget's disease pathophysiology

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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

On gross pathology, a plaque with an irregular border and erythematous or white skin lesion are characteristic findings of extramammary Paget's disease. On microscopic histopathological analysis, Paget's cells (large cells with abundant amphophilic or basophilic, finely granular cytoplasm, large centrally-located nucleus with 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. The neoplasm most likely to be associated with extramammary Paget's disease is an adnexal apocrine carcinoma, which usually represents infiltration of the deeper adnexa by epidermal Paget's cells.[1][2]

Pathophysiology

Gross Pathology

On gross pathology, the following are characteristic findings of extramammary Paget's disease:[2]

Microscopic Pathology

Pathogenesis

Immunohistochemistry

References

  1. 1.0 1.1 Roy J, Mirnezami A, Gatt M, Sasapu KK, Scott N, Sagar PM (2010). "A rare case of Paget's disease in a retrorectal dermoid cyst". Colorectal Dis. 12 (9): 946–7. doi:10.1111/j.1463-1318.2009.02102.x. PMID 19888952.
  2. 2.0 2.1 Ameloblastoma. Libre pathology(2015) http://librepathology.org/wiki/index.php/Extramammary_Paget_disease#Microscopic Accessed on January 30, 2016
  3. Lloyd, J (2000). "Mammary and extramammary Paget's disease". Journal of Clinical Pathology. 53 (10): 742–749. doi:10.1136/jcp.53.10.742. ISSN 0021-9746.
  4. Guarner J, Cohen C, DeRose PB (1989). "Histogenesis of extramammary and mammary Paget cells. An immunohistochemical study". Am J Dermatopathol. 11 (4): 313–8. PMID 2549798.
  5. Mazoujian G, Pinkus GS, Haagensen DE (1984). "Extramammary Paget's disease--evidence for an apocrine origin. An immunoperoxidase study of gross cystic disease fluid protein-15, carcinoembryonic antigen, and keratin proteins". Am J Surg Pathol. 8 (1): 43–50. PMID 6198933.
  6. Lloyd J, Flanagan AM (2000). "Mammary and extramammary Paget's disease". J Clin Pathol. 53 (10): 742–9. PMC 1731095. PMID 11064666.
  7. Jones, R. Russell; Spaull, J.; Gusterson, B. (1989). "The histogenesis of mammary and extramammary Paget's disease". Histopathology. 14 (4): 409–416. doi:10.1111/j.1365-2559.1989.tb02169.x. ISSN 0309-0167.
  8. 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.


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