Extramammary Paget's disease (EMPD) is a rare intraepithelial neoplasm arising in apocrine rich area of the skin. It is divided into primary and secondary EMPD based on whether there is an underlying malignancy, either local apocrine cancers or distant neoplasms. Therefore, all patients with EMPD should undergo an extensive and targeted cancer workup, depending on the histological staining pattern and the location. Surgical removal is considered the mainstay of treatment for patients with EMPD. Herein, we present a case of secondary EMPD where photodynamic therapy was effective in terms of improving symptoms and quality of life. Owing to preexisting comorbid conditions, large area of disease and risk of post-surgery mutilation and functional impairment, surgery could not be elected for this patient, necessitating nonsurgical approach.
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| http://dx.doi.org/10.1016/j.pdpdt.2018.04.002 | DOI Listing |
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The clinical and pathological significance of tertiary lymphoid structure in extramammary Paget's disease.
Front Immunol
December 2024
Department of Pathology, Shanghai Skin Disease Hospital, School of Medicine, Tongji University, Shanghai, China.
Background: Tumor-associated tertiary lymphoid structures (TLSs) are functional immune-responsive aggregates, which have been reported to be associated with better prognosis in various tumors. However, their exact characteristics and prognostic value in extramammary Paget's disease (EMPD) remain unknown.
Objective: To explore the features of TLSs in EMPD and their association with clinicopathological characteristics.
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Department of Urology, Emory University School of Medicine, Atlanta, GA, USA.
Extramammary Paget disease (EMPD) is a rare dermatologic malignancy with a high rate of recurrence and increased risk for developing secondary malignancies. We present a 74-year-old male with previously resected primary EMPD who presented with widespread PSMA-avid lesions without prostatic uptake, an elevated PSA >100, and a negative prostate biopsy. Based on this and immunohistological staining, recurrent EMPD was suspected.
View Article and Find Full Text PDFA Territory-Wide Follow-Up of Primary and Secondary Extramammary Paget Disease of 2 Decades: Effects of Local Disease on Survival.
Dermatology
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Department of Pathology, Queen Mary Hospital, The University of Hong Kong, Hong Kong, Hong Kong, China.
Introduction: Extramammary Paget disease (EMPD) is an uncommon malignant cutaneous neoplasm that are divided into primary and secondary forms. In this multicenter study, histologically proven cases of primary and secondary EMPD were reviewed for clinical outcomes with subgroup analysis for secondary EMPD.
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Article Synopsis
- Extramammary Paget's disease (EMPD) can be linked to underlying internal adenocarcinomas, with varying associations based on the EMPD subtype.
- A systematic literature review identified that perianal EMPD has a higher rate (25%) of associated adenocarcinomas compared to penoscrotal and vulvar types (6% each).
- The proposed screening algorithms suggest specific tests based on EMPD subtype, emphasizing thorough screening for high-risk perianal cases and more cost-effective approaches for lower-risk penoscrotal and vulvar cases.
Article Synopsis
- - Extramammary Paget's disease (EMPD) is a rare skin cancer classified as primary (without an internal malignancy) or secondary (linked to another cancer), with this case report detailing a patient who developed primary EMPD 10 years after having secondary EMPD in the same area.
- - The patient underwent a wide local excision for the secondary cervical EMPD, and subsequent analysis confirmed the emergence of primary EMPD, raising questions about the underlying causes like field cancerization or risk factors.
- - The report emphasizes the necessity of long-term follow-up care and the efficacy of Mohs micrographic surgery as the best treatment option for EMPD, highlighting the importance of careful diagnostics and monitoring for these patients.
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