Department of Pathology, Queen Elizabeth II Health Sciences Center, Nova Scotia Health Authority (Central Zone), Halifax, Nova Scotia, Canada.
Departments of Pathology and Medicine, Dalhousie University, Halifax, Nova Scotia, Canada.
J Cutan Pathol. 2021 Mar;48(3):411-421. doi: 10.1111/cup.13910. Epub 2020 Dec 3.
Merkel cell carcinoma has been a focus of active scientific investigation in recent years and new information on the topic has emerged. Although uncommon, this primary cutaneous neuroendocrine carcinoma, usually involving the head/neck of elderly individuals, has a poor prognosis. Within the past two decades, an increase in the incidence of the tumor and the discovery of its link to the Merkel cell polyomavirus have focused medical attention on the lesion. The resulting studies have improved our understanding of the biology of the neoplasm and contributed to clinical care. Specifically, two pathogenic subsets of the tumor have come to light, the majority due to Merkel cell polyomavirus and the minority caused by ultraviolet radiation-induced genetic damage. This dichotomy carries prognostic implications favoring the former subset. In addition, having capitalized on the known susceptibility of the tumor to immune influences, investigators have recently discovered its responsiveness to immune checkpoint inhibition. This revelation has constituted a therapeutic milestone at the clinical level. Herein we provide an overview of the topic, outline updates in the field and place an emphasis on dermatopathologic aspects of Merkel cell carcinoma.
近年来，默克尔细胞癌一直是活跃的科学研究的焦点，该领域出现了新的信息。虽然这种原发性皮肤神经内分泌癌并不常见，通常发生于老年患者的头颈部，但预后较差。在过去的二十年中，肿瘤发病率的增加和发现其与默克尔细胞多瘤病毒的关联引起了医学界对该病变的关注。由此产生的研究提高了我们对肿瘤生物学的理解，并有助于临床护理。具体而言，肿瘤的两个致病亚型已经出现，其中大多数是由默克尔细胞多瘤病毒引起的，少数是由紫外线辐射引起的遗传损伤引起的。这种二分法具有预后意义，有利于前一亚组。此外，研究人员利用肿瘤对免疫影响的已知易感性，最近发现其对免疫检查点抑制的反应性。这一发现构成了临床水平上的治疗里程碑。本文概述了这一主题，概述了该领域的最新进展，并重点介绍了默克尔细胞癌的皮肤病理方面。
