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Extramammary Paget's disease of the groin with underlying carcinoma and fatal outcome.
Clin Exp Dermatol 2008; 33(5):595-8CE

Abstract

Extramammary Paget's disease (EMPD) is considered to be an intraepithelial adenocarcinoma. Typically involved anatomical sites are the vulvar, perianal, perineal, scrotal and penile regions. Clinically, the lesions present as well-defined, moist, erythematous plaques usually accompanied by pruritus. An unusual feature of EMPD is its association with cutaneous, adnexal-structure adenocarcinomas and its association with internal malignancies. Histopathological examination shows epidermal acanthosis and elongated rete ridges. Paget's cells are large intraepidermal cells with a large nucleous and abundant pale cytoplasm. Recent studies of perianal and vulvar EMPD have described distinct immunohistochemical subtypes termed cutaneous and endodermal. Cutaneous EMPD is characteristically positive for cytokeratin (CK)7, negative for CK20, and positive for gross cystic disease fluid protein (GCDFP)15+, whereas endodermal EMPD shows a CK7+ CK20+ GCDFP15- phenotype. Surgery remains the treatment of choice, with either wide surgical excision or Mohs' micrographic surgery. We present a case of EMPD with an underlying carcinoma, which combined immunohistochemical findings suggestive of the cutaneous subtype (positive for CK7, GCDFP15, mucin (MUC)1, human epidermal growth factor receptor (HER)2/neu positive) and the endodermal subtype, frequently associated with internal malignancy (CK20, MUC2, CDX-2 positve); however, our patient had no associated internal malignancy.

Authors+Show Affiliations

Department of Dermatology, General University Hospital of Elche, Spain. pascual_josram@gva.esNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

18485020

Citation

Pascual, J C., et al. "Extramammary Paget's Disease of the Groin With Underlying Carcinoma and Fatal Outcome." Clinical and Experimental Dermatology, vol. 33, no. 5, 2008, pp. 595-8.
Pascual JC, Perez-Ramos M, Devesa JP, et al. Extramammary Paget's disease of the groin with underlying carcinoma and fatal outcome. Clin Exp Dermatol. 2008;33(5):595-8.
Pascual, J. C., Perez-Ramos, M., Devesa, J. P., Kutzner, H., & Requena, L. (2008). Extramammary Paget's disease of the groin with underlying carcinoma and fatal outcome. Clinical and Experimental Dermatology, 33(5), pp. 595-8. doi:10.1111/j.1365-2230.2008.02731.x.
Pascual JC, et al. Extramammary Paget's Disease of the Groin With Underlying Carcinoma and Fatal Outcome. Clin Exp Dermatol. 2008;33(5):595-8. PubMed PMID: 18485020.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Extramammary Paget's disease of the groin with underlying carcinoma and fatal outcome. AU - Pascual,J C, AU - Perez-Ramos,M, AU - Devesa,J P, AU - Kutzner,H, AU - Requena,L, Y1 - 2008/05/15/ PY - 2008/5/20/pubmed PY - 2008/12/17/medline PY - 2008/5/20/entrez SP - 595 EP - 8 JF - Clinical and experimental dermatology JO - Clin. Exp. Dermatol. VL - 33 IS - 5 N2 - Extramammary Paget's disease (EMPD) is considered to be an intraepithelial adenocarcinoma. Typically involved anatomical sites are the vulvar, perianal, perineal, scrotal and penile regions. Clinically, the lesions present as well-defined, moist, erythematous plaques usually accompanied by pruritus. An unusual feature of EMPD is its association with cutaneous, adnexal-structure adenocarcinomas and its association with internal malignancies. Histopathological examination shows epidermal acanthosis and elongated rete ridges. Paget's cells are large intraepidermal cells with a large nucleous and abundant pale cytoplasm. Recent studies of perianal and vulvar EMPD have described distinct immunohistochemical subtypes termed cutaneous and endodermal. Cutaneous EMPD is characteristically positive for cytokeratin (CK)7, negative for CK20, and positive for gross cystic disease fluid protein (GCDFP)15+, whereas endodermal EMPD shows a CK7+ CK20+ GCDFP15- phenotype. Surgery remains the treatment of choice, with either wide surgical excision or Mohs' micrographic surgery. We present a case of EMPD with an underlying carcinoma, which combined immunohistochemical findings suggestive of the cutaneous subtype (positive for CK7, GCDFP15, mucin (MUC)1, human epidermal growth factor receptor (HER)2/neu positive) and the endodermal subtype, frequently associated with internal malignancy (CK20, MUC2, CDX-2 positve); however, our patient had no associated internal malignancy. SN - 1365-2230 UR - https://www.unboundmedicine.com/medline/citation/18485020/Extramammary_Paget's_disease_of_the_groin_with_underlying_carcinoma_and_fatal_outcome_ L2 - https://doi.org/10.1111/j.1365-2230.2008.02731.x DB - PRIME DP - Unbound Medicine ER -