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<p>Merkel cell carcinoma (MCC) is a&#xa0;rare, highly aggressive neuroendocrine skin cancer with increasing incidence in aging and immunosuppressed populations. Current German and international guidelines recommend an interdisciplinary stage-adapted management approach with thorough imaging and sentinel lymph node biopsy. Approximately 80% of tumors in Europe are associated with Merkel cell polyomavirus, while the remainder are UV induced and display a&#xa0;high mutational burden. Poor prognostic factors include advanced stage, immunosuppression, and lack of viral association. The primary treatment/diagnostic measures for localized disease consist of complete excision with a&#xa0;surgical margin of 1–2 cm, complete imaging, sentinel lymph node biopsy, and risk-adapted adjuvant radiotherapy. For metastatic or inoperable, nonirradiable tumors, PD-1/PD-L1 inhibitors (retifanlimab and avelumab, respectively) are the treatment of choice, achieving durable responses in approximately half of the patients. Neoadjuvant and adjuvant immunotherapy approaches show promise and may further improve the prognosis of this historically unfavorable entity.</p>

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Merkel-Zell-Karzinom

  • 期刊论文

  • 发表日期：

    2026年5月28日

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  • Thilo Gambichler

摘要

Merkel cell carcinoma (MCC) is a rare, highly aggressive neuroendocrine skin cancer with increasing incidence in aging and immunosuppressed populations. Current German and international guidelines recommend an interdisciplinary stage-adapted management approach with thorough imaging and sentinel lymph node biopsy. Approximately 80% of tumors in Europe are associated with Merkel cell polyomavirus, while the remainder are UV induced and display a high mutational burden. Poor prognostic factors include advanced stage, immunosuppression, and lack of viral association. The primary treatment/diagnostic measures for localized disease consist of complete excision with a surgical margin of 1–2 cm, complete imaging, sentinel lymph node biopsy, and risk-adapted adjuvant radiotherapy. For metastatic or inoperable, nonirradiable tumors, PD-1/PD-L1 inhibitors (retifanlimab and avelumab, respectively) are the treatment of choice, achieving durable responses in approximately half of the patients. Neoadjuvant and adjuvant immunotherapy approaches show promise and may further improve the prognosis of this historically unfavorable entity.