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Cutaneous adnexal adenocarcinoma with exquisite sensitivity to trastuzumab
Timothy J Brown1, David J Sher2, Lucien A Nedzi2
1Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, Texas.
Background:
Cutaneous adnexal adenocarcinoma is a rare cancer that is occasionally human epidermal growth factor receptor-2 (HER-2)-positive, and demonstrates variable response to HER-2 inhibitors.
Methods:
We report a case of adnexal adenocarcinoma of the scalp in a 56-year-old man. He underwent wide local excision with cervical node dissection followed by radiation, but had extensive local recurrence.
Results:
Pathology demonstrated a poorly differentiated adnexal adenocarcinoma with HER-2 overexpression by immunohistochemistry (IHC) and high HER-2 gene amplification by fluorescence in situ hybridization. The patient was treated with trastuzumab-based therapy with dramatic response and clinical resolution of the tumor. Upon pausing trastuzumab, he developed local relapse, but had an excellent response to restarting trastuzumab monotherapy. He lacks visible disease 43 months after the initial diagnosis.
Conclusion:
We believe the exquisite sensitivity of the primary carcinoma and subsequent recurrence to trastuzumab therapy was due to strong HER-2 expression both at the protein and gene level. © 2017 Wiley Periodicals, Inc. Head Neck 39: E69-E71, 2017.
Insights
A rare scalp cancer (adnexal adenocarcinoma) that overexpressed human epidermal growth factor receptor-2 (HER2) responded dramatically to HER2-targeted therapy (trastuzumab), showing long-term remission.
Area of Science:
- Oncology
- Dermatology
- Molecular Biology
Background:
- Cutaneous adnexal adenocarcinoma is a rare malignancy.
- Occasionally, this cancer is human epidermal growth factor receptor-2 (HER2)-positive.
- Response to HER2 inhibitors can be variable.
Observation:
- A 56-year-old man presented with adnexal adenocarcinoma of the scalp.
- Despite surgery and radiation, he experienced extensive local recurrence.
- Pathology revealed HER2 overexpression and gene amplification.
Findings:
- The patient received trastuzumab-based therapy, achieving dramatic tumor response and clinical resolution.
- Upon pausing trastuzumab, local relapse occurred.
- Restarting trastuzumab monotherapy led to an excellent response, with no visible disease at 43 months.
Implications:
- Strong HER2 protein and gene expression correlated with exquisite sensitivity to trastuzumab.
- This case highlights the potential efficacy of HER2-targeted therapy in HER2-positive adnexal adenocarcinoma.
- Targeted therapy may offer a viable treatment option for this rare cancer.
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