Hormonal Environment and HER2 Status in Extra-Mammary Paget’s Disease (eMPD): A Systematic Literature Review and Meta-Analysis with Clinical Considerations
Giuseppe Angelico, Angela Santoro, Frediano Inzani, Patrizia Straccia, Damiano Arciuolo, Antonino Mulè, Michele Valente, Saveria Spadola, Nicoletta D’Alessandris, Giorgia Garganese, Federica Cianfrini, Alessia Piermattei, Giovanni Scambia, Gian Franco Zannoni
Abstract
BACKGROUND: Extra-mammary Paget's disease (EMPD) is a rare neoplasm of epithelial origin, whose precise incidence is not clear. Starting from what is already known, we performed a systematic review and meta-analysis to investigate in male and female patients the immunohistochemical expression of biological markers that could serve as potential prognostic/therapeutic factors, including only human epidermal growth factor receptor 2 (HER2/neu), Estrogen Receptor (ER), Progesterone Receptor (PR), and Androgen Receptor (AR). METHODS: A literature search was performed of the PubMed, Scopus, and Web of Science databases for English-language studies published from January 2000 to June 2020. RESULTS: A total of 27 studies with 713 patients assessed the role of HER2/neu, AR, ER, and PR expression in male and female with EMPD. The overall rate of HER2/neu expression was 30%, the expression's rate for ER and AR was 13% and 40%, respectively, and the overall rate for PR was 8%. The subgroup analysis revealed that there is a different expression of molecular markers between male and female patients. CONCLUSIONS: This study revealed that AR status and HER2/neu overexpression/amplification have been shown as two fundamental pathogenetic pathways in both female and male patients affected by EMPD.