Harms Paul W
Department of Pathology, University of Michigan Medical School, 3261 Medical Science I, 1301 Catherine Street, Ann Arbor, MI 48109-5602, USA; Department of Dermatology, University of Michigan Medical School, 3261 Medical Science I, 1301 Catherine Street, Ann Arbor, MI 48109-5602, USA.
Clin Lab Med. 2017 Sep;37(3):485-501. doi: 10.1016/j.cll.2017.05.004. Epub 2017 Jun 13.
Merkel cell carcinoma (MCC) is a rare, aggressive cutaneous neuroendocrine malignancy. Merkel cell polyomavirus, a tumorigenic DNA virus, is present in most MCC tumors, with implications for tumor biology, diagnosis, and management. Merkel cell polyomavirus-negative tumors have a high burden of UV-signature mutations, similar to melanoma. The histopathologic diagnosis of MCC requires immunohistochemistry to exclude morphologically similar entities. Therapies for advanced disease are currently lacking. Here, the features of MCC are reviewed, including recent molecular discoveries with implications for improved therapy for advanced disease.
默克尔细胞癌(MCC)是一种罕见的、侵袭性皮肤神经内分泌恶性肿瘤。默克尔细胞多瘤病毒是一种致瘤性DNA病毒,存在于大多数MCC肿瘤中,对肿瘤生物学、诊断和治疗具有重要意义。默克尔细胞多瘤病毒阴性肿瘤具有高负荷的紫外线特征性突变,类似于黑色素瘤。MCC的组织病理学诊断需要免疫组织化学来排除形态学上相似的实体。目前缺乏针对晚期疾病的治疗方法。在此,对MCC的特征进行综述,包括最近的分子发现及其对改善晚期疾病治疗的意义。