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Successful human epidermal growth receptor 2-targeted therapy beyond disease progression for extramammary Paget's
Satomi Watanabe1, Masayuki Takeda2, Takayuki Takahama1
1Department of Medical Oncology, Kinki University Faculty of Medicine, 377-2 Ohno-higashi, Osaka-Sayama, Osaka, 589-8511, Japan.
Abstract:
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.
Insights
Extramammary Paget's disease treatment shows promise. Trastuzumab, targeting human epidermal growth factor receptor 2, combined with docetaxel and pertuzumab, sustained a patient's response for 12 months.
Area of Science:
- Oncology
- Dermatology
- Medical Research
Background:
- Extramammary Paget's disease is a rare intraepithelial carcinoma, often affecting the vulva.
- Surgical resection is primary, but advanced/recurrent cases lack established chemotherapy.
- Human Epidermal Growth Factor Receptor 2 (HER2) is a potential therapeutic target.
Observation:
- A 63-year-old male presented with recurrent EMPD post-surgery.
- Immunohistochemistry and FISH confirmed HER2 overexpression and amplification in the tumor.
- Initial trastuzumab monotherapy reduced lymph node metastases but led to recurrence.
Findings:
- Trastuzumab monotherapy showed initial efficacy in reducing lymph node metastases.
- Recurrence occurred despite ongoing trastuzumab treatment.
- Combination therapy with trastuzumab, docetaxel, and pertuzumab achieved sustained clinical response for 12 months.
Implications:
- HER2-targeted therapy, including combination regimens, is a viable strategy for recurrent EMPD.
- This approach offers a new treatment avenue for patients with advanced or refractory disease.
- Further research into HER2-driven EMPD could optimize treatment protocols.
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