Division of Gastrointestinal Medical Oncology and Neuroendocrine Tumors, European Institute of Oncology (IEO) IRCCS, Milano, Italy.
Medical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences and Public Health-Medical Oncology, University of Brescia, ASST-Spedali Civili, Brescia, Italy.
J Immunother Cancer. 2022 Jun;10(6). doi: 10.1136/jitc-2022-004742.
Merkel cell carcinoma (MCC) is a rare and highly aggressive cutaneous neuroendocrine carcinoma. The MCC incidence rate has rapidly grown over the last years, with Italy showing the highest increase among European countries. This malignancy has been the focus of active scientific research over the last years, focusing mainly on pathogenesis, new therapeutic trials and diagnosis. A national expert board developed 28 consensus statements that delineated the evolution of disease management and highlighted the paradigm shift towards the use of immunological strategies, which were then presented to a national MCC specialists panel for review. Sixty-five panelists answered both rounds of the questionnaire. The statements were divided into five areas: a high level of agreement was reached in the area of guidelines and multidisciplinary management, even if in real life the multidisciplinary team was not always represented by all the specialists. In the diagnostic pathway area, imaging played a crucial role in diagnosis and initial staging, planning for surgery or radiation therapy, assessment of treatment response and surveillance of recurrence and metastases. Concerning diagnosis, the usefulness of Merkel cell polyomavirus is recognized, but the agreement and consensus regarding the need for cytokeratin evaluation appears greater. Regarding the areas of clinical management and follow-up, patients with MCC require customized treatment. There was a wide dispersion of results and the suggestion to increase awareness about the adjuvant radiation therapy. The panelists unanimously agreed that the information concerning avelumab provided by the JAVELIN Merkel 200 study is adequate and reliable and that the expanded access program data could have concrete clinical implications. An immunocompromised patient with advanced MCC can be treated with immunotherapy after multidisciplinary risk/benefit assessment, as evidenced by real-world analysis and highlighted in the guidelines. A very high consensus regarding the addition of radiotherapy to treat the ongoing focal progression of immunotherapy was observed. This paper emphasizes the importance of collaboration and communication among the interprofessional team members and encourages managing patients with MCC within dedicated multidisciplinary teams. New insights in the treatment of this challenging cancer needs the contribution of many and different experts.
默克尔细胞癌(Merkel cell carcinoma,MCC)是一种罕见且高度侵袭性的皮肤神经内分泌癌。近年来,MCC 的发病率迅速上升,意大利是欧洲国家中发病率增长最快的国家。近年来,这种恶性肿瘤一直是积极的科学研究的焦点,主要集中在发病机制、新的治疗试验和诊断上。一个国家专家委员会制定了 28 项共识声明,勾勒出疾病管理的演变,并强调了向免疫策略转变的范例,然后提交给一个国家的 MCC 专家小组进行审查。65 名小组成员回答了两轮问卷调查。这些声明分为五个领域:在指南和多学科管理领域达成了高度一致,即使在现实生活中,多学科团队并不总是由所有专家组成。在诊断途径领域,影像学在诊断和初始分期、手术或放射治疗计划、评估治疗反应以及监测复发和转移方面发挥了关键作用。关于诊断,已经认识到 Merkel 细胞多瘤病毒的有用性,但对于需要进行细胞角蛋白评估的共识和一致性似乎更大。关于临床管理和随访领域,MCC 患者需要定制治疗。结果存在广泛差异,并建议提高对辅助放射治疗的认识。小组成员一致认为,JAVELIN Merkel 200 研究提供的avelumab 信息是充分和可靠的,扩大准入计划的数据可能具有具体的临床意义。经过多学科风险/获益评估,免疫功能低下的晚期 MCC 患者可以接受免疫治疗,这一点已经被真实世界的分析所证实,并在指南中得到强调。观察到对于用放射治疗来治疗免疫治疗中持续的局部进展非常高的一致性。本文强调了跨专业团队成员之间合作和沟通的重要性,并鼓励在专门的多学科团队中管理 MCC 患者。治疗这种具有挑战性的癌症的新见解需要许多不同专家的共同努力。