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Merkel 细胞癌:发病机制、诊断和治疗选择的最新综述。

Merkel cell carcinoma: An updated review of pathogenesis, diagnosis, and treatment options.

机构信息

Dr. Phillip Frost Department of Dermatology and Cutaneous Surgery, Miller School of Medicine, University of Miami, Miami, Florida, USA.

Department of Hematology-Oncology, Maroone Cancer Center, Cleveland Clinic Florida, Weston, Florida, USA.

出版信息

Dermatol Ther. 2022 Mar;35(3):e15292. doi: 10.1111/dth.15292. Epub 2022 Jan 5.

Abstract

Merkel cell carcinoma is a rare neuroendocrine carcinoma that typically appears in sun-exposed areas of the elderly. It has a poor prognosis and with its incidence projected to increase, it is vital for dermatologists to remain up to date with recent updates in this malignancy's pathogenesis and treatment. In the past few decades Merkel cell carcinoma's pathogenesis, more specifically its relation to the Merkel cell polyomavirus, has sparked further interest in the study of this carcinoma. Most cases are attributed to malignant transformation secondary to the Merkel cell polyomavirus, with a minority derived from DNA damage resulting from ultraviolet radiation. Investigators have also determined that there are immunologic influences in the development and prognosis of Merkel cell carcinoma, as individuals with HIV, solid organ transplants, and lymphoproliferative malignancies are at a greater risk of developing this carcinoma. In addition, this immunologic link carries treatment value, as immunologic therapies are currently being investigated. This article provides a comprehensive review of the epidemiology and pathogenesis of Merkel cell carcinoma as well as the current treatments available and clinical trials underway. We also touch upon the updated National Comprehensive Cancer Network Clinical Practice Guidelines in Oncology in respect to its diagnosis and recommended treatment modalities.

摘要

默克尔细胞癌是一种罕见的神经内分泌癌,通常出现在老年人暴露于阳光的部位。它的预后很差,由于预计发病率会增加,皮肤科医生了解这种恶性肿瘤的发病机制和治疗的最新进展至关重要。在过去的几十年里,默克尔细胞癌的发病机制,特别是它与默克尔细胞多瘤病毒的关系,激发了人们对这种癌症的进一步研究兴趣。大多数病例归因于默克尔细胞多瘤病毒引起的恶性转化,少数病例源于紫外线辐射引起的 DNA 损伤。研究人员还确定,在默克尔细胞癌的发生和预后中存在免疫影响,因为 HIV 感染者、实体器官移植受者和淋巴增生性恶性肿瘤患者患这种癌症的风险更高。此外,这种免疫联系具有治疗价值,因为目前正在研究免疫疗法。本文全面回顾了默克尔细胞癌的流行病学和发病机制,以及目前可用的治疗方法和正在进行的临床试验。我们还谈到了最新的国家综合癌症网络肿瘤学临床实践指南在其诊断和推荐的治疗方式方面的内容。

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