HER2/neu

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A novel therapeutic option for HER2-positive extramammary Paget disease

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A novel therapeutic option for HER2-positive extramammary Paget disease

The research article evaluates HER2 status in extramammary Paget disease (EMPD) and explores the use of disitamab vedotin (DV), an anti-HER2 antibody-drug conjugate, as a treatment. It found significant HER2 expression in a substantial proportion of EMPD cases, with higher expression linked to more aggressive disease and lymph node metastasis. In two advanced EMPD patients resistant to chemotherapy, DV showed partial responses, suggesting that HER2-targeted therapy could be effective even in cases with low HER2 levels. The study highlights the potential of HER2-targeted treatments and calls for further research into their efficacy in EMPD.

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Whole genome sequencing of HER2-positive metastatic extramammary Paget’s disease

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Whole genome sequencing of HER2-positive metastatic extramammary Paget’s disease

Extramammary Paget’s disease (EMPD) is a rare cancer that occurs within the epithelium of the skin, arising predominantly in areas with high apocrine gland concentration such as the vulva, scrotum, penis and perianal regions. Here, we aim to integrate clinicopathological data with genomic analysis of aggressive, rapidly-progressing de novometastatic EMPD responding to HER2-directed treatment in combination with other agents, to attain a more comprehensive understanding of the disease landscape

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Long-Term Complete Response and Survival in Metastatic Extramammary Paget’s Disease Treated With Trastuzumab Plus Paclitaxel: A Case Report

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Long-Term Complete Response and Survival in Metastatic Extramammary Paget’s Disease Treated With Trastuzumab Plus Paclitaxel: A Case Report

Extramammary Paget’s disease is a rare skin cancer that usually arises from the secretory cells of the apocrine glands. In most cases, an extramammary Paget’s tumor occurs as a single intraepithelial form not associated with another cancer, although rarely, it may be associated with other loco-regional or distant cancer. In this case report, we have observed a long-lasting complete response with anti-HER2 plus paclitaxel.

Hormonal Environment and HER2 Status in Extra-Mammary Paget’s Disease (EMPD)

Hormonal Environment and HER2 Status in Extra-Mammary Paget’s Disease (EMPD)

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.

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