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Reviewing vulvar Paget's disease molecular bases. Looking forward to personalized target therapies: a matter of
Giulia Mantovani1, Anna Fagotti2,3, Massimo Franchi4
1Department of Obstetrics and Gynecology, Università degli Studi di Verona, Verona, Italy giuliamanto86@gmail.com.
Objectives:
To review the published literature on vulvar Paget's disease (VPD) molecular bases, aiming to support the need for tailored treatment in women affected by this 'orphan' tumor.
Methods:
MEDLINE-PubMed and Scopus were interrogated using the following algorithm: (extramammary OR extra mammary OR vulvar) AND (paget OR pagets OR paget's) AND (molecular OR biological OR marker OR protein OR target OR expression). The inclusion criteria for papers were: peer-reviewed English-language journals, articles published in the last 30 years, studies focused on fixed research questions, quality assessment on the basis of the relevance and contribution to the selected topics.
Results:
A total of 42 studies were selected, providing the following results. Molecular markers implicated in cell cycle transitions seem to be related to prognosis and could help to tailor conventional treatments. Fragmented but consistent preliminary data exist on hormonal receptor expression, ERBB2 amplification/overexpression and abnormal vascular proliferation, offering a concrete possibility for target therapy trials. Conversely, other fields linked to the possible use of immunotherapy are currently relatively unexplored, such as the tumor 'immune contexture', programmed death ligand-1 (PD-L1) expression and defects in the mismatch repair system, which is involved in genomic instability and potentially promotes a consistent response to treatment.
Conclusions:
Additional effort is needed to further characterize these aspects. Centralization of patients in dedicated units would be beneficial for concentrating patient numbers, collecting valuable clinical data and conducting clinical trials. Interdisciplinary study platforms should be developed and integrated into wider multicentric networks.
Insights
This review highlights molecular markers in vulvar Paget's disease (VPD) that could guide targeted therapies. Further research and centralized patient data are crucial for developing effective treatments for this rare cancer.
Area of Science:
- Oncology
- Molecular Biology
- Dermatology
Background:
- Vulvar Paget's disease (VPD) is a rare gynecological malignancy.
- Understanding the molecular underpinnings of VPD is essential for developing targeted therapies.
- VPD is often considered an 'orphan' tumor, necessitating specialized research and treatment approaches.
Purpose of the Study:
- To review the published literature on the molecular bases of vulvar Paget's disease.
- To identify molecular markers that can inform tailored treatment strategies for VPD.
- To highlight the need for further research and clinical trials in VPD.
Main Methods:
- A systematic literature search was conducted using MEDLINE-PubMed and Scopus.
- Search terms included "extramammary," "Paget's," and "molecular" related keywords.
- Inclusion criteria focused on peer-reviewed, English-language articles from the last 30 years addressing specific research questions.
Main Results:
- Forty-two studies were selected for review.
- Molecular markers associated with cell cycle regulation may predict prognosis and guide conventional treatments.
- Preliminary data suggest potential for targeted therapies based on hormonal receptor expression, ERBB2 amplification/overexpression, and abnormal vascular proliferation.
- Immunotherapy targets, including PD-L1 expression and mismatch repair defects, are underexplored but hold promise.
Conclusions:
- Further characterization of molecular aspects of VPD is required.
- Centralizing patients in dedicated units can facilitate data collection and clinical trials.
- Development of interdisciplinary study platforms within multicentric networks is recommended.