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Case Reports
. 2020 May 18;12(2):107-113.
doi: 10.1159/000507917. eCollection 2020 May-Aug.

Extramammary Paget's Disease and Melanoma: 2 Cases of Double Cancers

Erika Lydrup  1 , Jais Oliver Berg  1 , Sofie Vetli Hjorth  2 , Lisbet Rosenkrantz Hølmich  1 , Jørgen Lock-Andersen  1 , Annette Hougaard Chakera  1
Affiliations
Case Reports

Extramammary Paget's Disease and Melanoma: 2 Cases of Double Cancers

Erika Lydrup et al. Case Rep Dermatol. .

Abstract

Extramammary Paget's disease (EMPD) is a rare intraepidermal neoplastic disease. There is a well-known relationship between EMPD and underlying malignancy. However, only a few cases of EMPD and cutaneous melanoma have been reported previously. In this case report we present 2 cases of such double cancers: one as a collision tumor, the other at separate sites. We discuss the pathogenesis, treatment, and importance of a thorough clinical and radiological examination and review the literature.

Keywords: Double cancer; Extramammary Paget's disease; Melanoma.

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Conflict of interest statement

The authors have no conflicts of interest to disclose.

Figures

Fig. 1
Fig. 1
a–d Histological presentation of the perianal skin showing Paget cells located in the epidermis. a Neoplastic cells with abundant cytoplasm and large vesicular nuclei with prominent nucleoli are arranged in confluent nests and as single cells throughout the epidermis. Hematoxylin-eosin. ×20. Tumor cells were negative for CK5 (b), but the cells stained for CK7 (c). d Tumor cells express Ber-EP4, allowing the diagnosis of extramammary Paget disease. Immunohistological staining. Magnification, ×10. The cells were negative for Sox10 (immunohistological stain).
Fig. 2
Fig. 2
Histological presentation of postauricular skin next to the melanoma. Paget cells are located in the epidermis and in the adnexal epithelium in the dermis. a Pagetoid distribution of Paget cells in the epidermis. Hematoxylin-eosin. ×20. b Tumor cells were negative for P40, while normal epithelium was positive. c Cells stained for CK7. Immunohistological stains. Magnification, ×10. d Sox10 staining of invasive melanoma. Immunohistological stain. Magnification, ×10. Immunohistological stains: CK7, cytokeratin 7; P40, protein 40; Ber-EP4, epithelial cell adhesion molecule; Sox10, Sox10 protein.
Fig. 3
Fig. 3
Clinical photograph of the lesion behind the left ear. Biopsy showed MM and EMPD in the same area.
See this image and copyright information in PMC

References

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