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Successful human epidermal growth receptor 2-targeted therapy beyond disease progression for extramammary Paget’s disease

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Summary

Extramammary Paget’s disease is a malignant intraepithelial carcinoma, which constitutes less than 1 % of all vulvar malignancies. Surgical resection is the first treatment of choice and standard chemotherapy has not been established for advanced or recurrent disease. Experimental and clinical studies have identified human epidermal growth receptor 2 as a potential therapeutic target. A 63-year-old male was referred for recurrent extramammary Paget’s disease after surgery. Human epidermal growth receptor 2 was shown to be overexpressed and amplified by immunohistochemical analysis and fluorescence in situ hybridization analysis, respectively. After two cycles of trastuzumab monotherapy, all lymph node metastases decreased in size. However, he experienced recurrence in the lymph nodes during the seven courses of trastuzumab. As a subsequent treatment, trastuzumab was administered in combination with docetaxel and pertuzumab; clinical response was sustained for 12 months without significant adverse events.

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Acknowledgments

This study was supported in part by JSPS KAKENHI Grant Number 15 K21525, Uehara Memorial Foundation, and Takeda Science Foundation.

Authors’ contributions

SW and MT were responsible for clinical management of the patient, acquisition of data, and drafting the manuscript; TI, TT, JT, YW, NI, JT, TS, KS, KN and KN were responsible for interpretation of data and critical revision of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Masayuki Takeda.

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Written informed consent was obtained from the patient for publication of this case report and accompanying images before the patient received chemotherapy.

Conflict Interest

Kazuhiko Nakagawa received honoraria from Chugai pharmaceuticals.

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Watanabe, S., Takeda, M., Takahama, T. et al. Successful human epidermal growth receptor 2-targeted therapy beyond disease progression for extramammary Paget’s disease. Invest New Drugs 34, 394–396 (2016). https://doi.org/10.1007/s10637-016-0329-8

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  • DOI: https://doi.org/10.1007/s10637-016-0329-8

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