Extramammary Paget Disease Management and Therapeutics

Extramammary Paget disease (EMPD) is a rare cutaneous adenocarcinoma arising predominantly in apocrine gland–bearing regions such as the vulva, scrotum and perianal area. Clinically it presents as erythematous, often pruritic plaques that mimic benign dermatological conditions, leading to delayed diagnosis. The mainstay of management remains surgical excision with clear margins, yet recurrence rates are high and margin determination can be challenging. Adjuvant radiotherapy or topical agents such as imiquimod and photodynamic therapy offer alternatives in patients unfit for extensive surgery. In advanced or metastatic EMPD, systemic options have historically been limited to conventional chemotherapy with modest success. Recognition of Her2 overexpression in a substantial subset of cases has prompted off-label use of anti-Her2 antibodies and antibody-drug conjugates. Ongoing molecular profiling efforts aim to identify actionable targets beyond Her2, while advances in intraoperative margin assessment and immunotherapy combinations hold promise for improving outcomes in both localised and disseminated disease.

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Extramammary Paget Disease Management and Therapeutics publication trend

The graph below shows the total number of articles in extramammary paget disease management and therapeutics across all publications each year (not limited to Nature Index journals).

Technical terms

Extramammary Paget disease (EMPD): A rare intraepithelial adenocarcinoma arising in apocrine gland–rich skin outside the breast, presenting as eczematous plaques.

Antibody-drug conjugate (ADC): A targeted therapeutic combining a monoclonal antibody with a cytotoxic payload to deliver chemotherapy directly to tumour cells expressing a specific antigen.

Fluorescence lifetime imaging microscopy (FLIM): An imaging modality that measures the decay time of fluorescent signals in tissue, enabling differentiation of tumour from normal margins when coupled with computational analysis.

NECTIN4: A cell adhesion molecule overexpressed in various epithelial cancers; its presence in EMPD suggests a potential target for ADC-based therapies.

References

1. Case report: Disitamab vedotin combined with immunotherapy demonstrated excellent efficacy in scrotal Paget’s disease with Her-2 overexpression. Frontiers in Immunology (2024).
2. Rapid and precise multifocal cutaneous tumor margin assessment using fluorescence lifetime detection and machine learning. APL Photonics (2024).
3. NECTIN4 Expression in Extramammary Paget’s Disease: Implication of a New Therapeutic Target. International Journal of Molecular Sciences (2020).