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Review
. 2023 Oct;25(10):1081-1094.
doi: 10.1007/s11912-023-01434-0. Epub 2023 Jul 8.

Extramammary Paget Disease: a Therapeutic Challenge, for a Rare Entity

Jesús Chamorro Pérez  1 , Alfonso Cortes Salgado  1 , Belén Pérez-Mies  2 , Jose Antonio Domínguez Rullán  3 , Odile Ajuria-Illarramendi  4 , Eva María Guerra Alia  1 , Juan José Serrano Domingo  5
Affiliations
Review

Extramammary Paget Disease: a Therapeutic Challenge, for a Rare Entity

Jesús Chamorro Pérez et al. Curr Oncol Rep. 2023 Oct.

Erratum in

Abstract

Purpose of review: Extramammary Paget disease (EMPD) is a rare entity which is more frequently localized at the vulva, though it only accounts for 1-2% of vulvar neoplasms. It is a primary cutaneous adenocarcinoma whose cell of origin is still a matter of controversy: it can either arise from apocrine/eccrine glands or from stem cells. The diagnosis demands a biopsy and entails a histopathological analysis by which cells show similar characteristics as breast Paget disease.

Recent findings: Treatment approach can entail surgery, radiotherapy, photodynamic therapy, systemic chemotherapy, and topical chemotherapy. For metastatic disease, many different chemotherapy regimens have been explored and even targeted therapy can play an important role in this disease. Since almost 30-40% of patients overexpress HER-2, trastuzumab and anti-HER-2 therapies can be employed in this setting. Due to its low incidence, there is almost no specific evidence on therapeutic interventions for this disease. Thus, there is a neat unmet need for molecular characterization of EMPD and diagnostic tools that allow clinicians to guide treatment both in the early and in the advanced disease settings. In this review, we aim to summarize available evidence about diagnosis and treatment of EMPD, both localized and metastatic, and to provide a comprehensive analysis that may help clinicians for therapeutic decisions.

Keywords: Chemotherapy; Extramammary Paget disease; HER-2; Imiquimod; Radiotherapy.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
a Paget disease (hematoxylin and eosin): nests of pale-staining tumor cells are arranged singly or in small cluster along the vulvar epidermis. b CK7 immunohistochemistry in a Paget disease
Fig. 2
Fig. 2
a TAC image with nodal affection. b PET image with nodal affection
Fig. 3
Fig. 3
a Paget disease (hematoxylin and eosin): nests of tumoral cells with pale cytoplasm infiltrating the vulvar dermis. b HER-2 immunohistochemistry (HercepTest, Agilent) scored as positive 3+
See this image and copyright information in PMC

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