Successful human epidermal growth receptor 2-targeted therapy beyond disease progression for extramammary Paget’s disease
- 350 Downloads
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.
KeywordsExtramammary Paget’s disease HER2 Molecular targeted therapy
This study was supported in part by JSPS KAKENHI Grant Number 15 K21525, Uehara Memorial Foundation, and Takeda Science Foundation.
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.
Compliance with ethical standards
Written informed consent was obtained from the patient for publication of this case report and accompanying images before the patient received chemotherapy.
Kazuhiko Nakagawa received honoraria from Chugai pharmaceuticals.
- 8.Barth P, Dulaimi Al-Saleem E, Edwards KW et al (2015) Metastatic extramammary Paget’s disease of scrotum responds completely to single agent Trastuzumab in a hemodialysis patient: case report molecular profiling and brief review of the literature. Case Rep Oncol Med 2015:895151PubMedPubMedCentralGoogle Scholar