Pan Kelly, Vromans A Maria, Cheng Liang, Grant-Kels Jane M, Katz Steven C, Hadfield Matthew J
Department of Internal Medicine, Brown University Health, Providence, RI, USA.
Department of Pathology and Laboratory Medicine, Department of Surgery (Urology), Brown University Warren Alpert Medical School, Legorreta Cancer Center at Brown University, Brown University Health, Providence, RI, USA.
Curr Oncol Rep. 2025 Jun 3. doi: 10.1007/s11912-025-01693-z.
Merkel cell carcinoma (MCC) is a rare and aggressive cutaneous neuroendocrine carcinoma that is primarily driven by Merkel cell polyomavirus (MCPyV) and ultraviolet radiation. Due to its rarity and innocuous appearance on clinical exam, MCC diagnosis is often delayed and therefore diagnosed at advanced stages. Overall survival outcomes are poor and notably worse than melanoma, with an estimated five-year survival ranging from 35 to 60% for stage I or II disease to < 15% for metastatic disease. Our review examines the diagnostic workup, prognostic markers, and current and emerging treatments of MCC.
For local disease in which surgical resection is feasible, tumor removal with potential adjuvant radiation therapy is the primary treatment modality. Immunotherapy with PD-1 / PD-L1 inhibitors is now standard for advanced disease where complete resection is not feasible. Additionally, there are many ongoing clinical trials examining novel immune checkpoint inhibitors, immunomodulators, targeted therapies, cellular therapies, vaccines, and oncolytic virus therapies with the goal of improving outcomes for patients with advanced disease or those who experience recurrence after first-line immunotherapy. MCC is an aggressive disease with a rapidly evolving treatment landscape, and emerging therapies hold the potential to improve prognosis in advanced disease.
默克尔细胞癌（MCC）是一种罕见且侵袭性强的皮肤神经内分泌癌，主要由默克尔细胞多瘤病毒（MCPyV）和紫外线辐射驱动。由于其罕见性以及在临床检查中外观无害，MCC的诊断常常延迟，因此多在晚期才得以确诊。总体生存结果较差，明显比黑色素瘤更糟，I期或II期疾病的估计五年生存率为35%至60%，而转移性疾病的五年生存率则低于15%。我们的综述探讨了MCC的诊断检查、预后标志物以及当前和新兴的治疗方法。
对于可行手术切除的局部疾病，手术切除并可能辅以放射治疗是主要的治疗方式。对于无法进行完全切除的晚期疾病，使用PD-1/PD-L1抑制剂进行免疫治疗现已成为标准治疗方法。此外，有许多正在进行的临床试验正在研究新型免疫检查点抑制剂、免疫调节剂、靶向治疗、细胞治疗、疫苗和溶瘤病毒疗法，目的是改善晚期疾病患者或一线免疫治疗后复发患者的治疗效果。MCC是一种侵袭性疾病，治疗格局迅速演变，新兴疗法有望改善晚期疾病的预后。
