Department of Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland.
Faculty of Medicine, Medical University of Warsaw, 02-091 Warsaw, Poland.
Int J Mol Sci. 2021 Jun 11;22(12):6305. doi: 10.3390/ijms22126305.
Merkel cell carcinoma (MCC) is an uncommon and highly aggressive skin cancer. It develops mostly within chronically sun-exposed areas of the skin. MCPyV is detected in 60-80% of MCC cases as integrated within the genome and is considered a major risk factor for MCC. Viral negative MCCs have a high mutation burden with a UV damage signature. Aberrations occur in , , and genes as well as in the PI3K-AKT-mTOR pathway. MCC is highly immunogenic, but MCC cells are known to evade the host's immune response. Despite the characteristic immunohistological profile of MCC, the diagnosis is challenging, and it should be confirmed by an experienced pathologist. Sentinel lymph node biopsy is considered the most reliable staging tool to identify subclinical nodal disease. Subclinical node metastases are present in about 30-50% of patients with primary MCC. The basis of MCC treatment is surgical excision. MCC is highly radiosensitive. It becomes chemoresistant within a few months. MCC is prone to recurrence. The outcomes in patients with metastatic disease are poor, with a historical 5-year survival of 13.5%. The median progression-free survival is 3-5 months, and the median overall survival is ten months. Currently, immunotherapy has become a standard of care first-line therapy for advanced MCC.
默克尔细胞癌（Merkel cell carcinoma，MCC）是一种罕见且高度侵袭性的皮肤癌。它主要发生在皮肤长期暴露于阳光的区域。MCPyV 存在于 60-80%的 MCC 病例中，作为整合在基因组内的病毒，被认为是 MCC 的主要危险因素。病毒阴性的 MCC 具有高突变负担和 UV 损伤特征。,, 和 基因以及 PI3K-AKT-mTOR 通路发生异常。MCC 具有高度免疫原性，但 MCC 细胞已知会逃避宿主的免疫反应。尽管 MCC 具有特征性的免疫组织化学特征，但诊断具有挑战性，应由经验丰富的病理学家确认。前哨淋巴结活检被认为是识别临床前淋巴结疾病最可靠的分期工具。约 30-50%的原发性 MCC 患者存在临床前淋巴结转移。MCC 治疗的基础是手术切除。MCC 对放射治疗高度敏感。在几个月内就会产生化疗耐药性。MCC 容易复发。转移性疾病患者的预后较差，历史 5 年生存率为 13.5%。无进展生存期的中位数为 3-5 个月，总生存期的中位数为 10 个月。目前，免疫疗法已成为晚期 MCC 的标准一线治疗方法。
