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Extramammary Paget's Disease in Two Brothers
Xiaoting Zhang1, Wanwan Jin1, Haibo Zhu2
1Department of Dermatology, The second Affiliated Hospital and Yuying children's hospital of Wenzhou Medical University, Wenzhou, Zhejiang 325000, People's Republic of China.
Indian Journal of Dermatology
|August 20, 2015
Summary
Extramammary Paget's disease (EMPD) is a rare cancer. Surgical resection is standard for local EMPD, while advanced cases benefit from targeted therapies like Trastuzumab when HER2 is overexpressed.
Area of Science:
- Oncology
- Dermatology
- Genetics
Background:
- Extramammary Paget's disease (EMPD) is a rare apocrine gland adenocarcinoma.
- Local EMPD is treated with surgical resection; advanced EMPD lacks effective treatments.
- Trastuzumab targets human epidermal growth factor receptor 2 (HER2) and shows efficacy in HER2-overexpressing advanced EMPD.
Purpose of the Study:
- To report two cases of EMPD in brothers.
- To illustrate treatment approaches for local and advanced EMPD.
- To highlight the potential of Trastuzumab in advanced EMPD.
Main Methods:
- Case report of two brothers with EMPD.
- Surgical resection for local disease.
- Combination chemotherapy and Trastuzumab for advanced, metastatic EMPD.
Main Results:
- The younger brother with local EMPD had successful surgical resection and remained recurrence-free for 15 months.
- The older brother with invasive EMPD and metastatic lymph nodes received chemotherapy and Trastuzumab post-surgery.
- This case series demonstrates varied therapeutic outcomes based on disease stage.
Conclusions:
- Surgical resection remains a viable option for localized EMPD.
- Advanced EMPD requires multimodal treatment, including chemotherapy and targeted therapy.
- HER2-targeted therapy with Trastuzumab shows promise for advanced EMPD with HER2 overexpression.
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