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首次原发性诊断21年后出现的晚期继发性默克尔细胞癌:何时降低监测强度?

Late subsequent Merkel cell carcinoma after 21 years of the first primary diagnosis: When to de-escalate surveillance?

作者信息

Borda Luis J, Cushman Courtny S, Pariser Robert J

机构信息

Department of Dermatology, Eastern Virginia Medical School Norfolk Virginia USA.

School of Medicine, Eastern Virginia Medical School Norfolk Virginia USA.

出版信息

Clin Case Rep. 2024 Aug 15;12(8):e9340. doi: 10.1002/ccr3.9340. eCollection 2024 Aug.

DOI:10.1002/ccr3.9340
PMID:39156199
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11327293/
Abstract

KEY CLINICAL MESSAGE

Merkel cell carcinoma (MCC) presents challenges in surveillance due to varied recurrence rates and uncertain follow-up protocols, especially in late recurrent cases. These cases need personalized monitoring strategies beyond traditional timelines, such as clinical and molecular factors, in order to optimize patient outcomes.

ABSTRACT

Merkel cell carcinoma (MCC) is a rare, aggressive skin cancer with neuroendocrine differentiation with a propensity for recurrence following initial treatment. Surveillance strategies for MCC patients lack specificity, and the duration of surveillance remains uncertain, posing challenges in identifying appropriate follow-up intervals. Therefore, we present a 94-year-old woman, with history of stage IA MCC in her left nasal wall 21 years prior, that presented with a dome-shaped eroded nodule on her left fifth finger. Biopsy showed characteristic MCC features with positive immunohistochemistry for CD56, synaptophysin, and CK20 (perinuclear dotting). The patient opted against further imaging or lymph node biopsy and underwent Mohs micrographic surgery. To date, there has not been any evidence of recurrence at previous sites or development of new primary lesions. This case underscores the need for ongoing surveillance despite long disease-free intervals. It also stands out as the case demonstrating the longest latency/recurrence-free interval following the initial diagnosis of MCC in the literature. While most recurrences occur within the first few years post-diagnosis, our case highlights the exceptional nature of late recurrences and prompts reevaluation of surveillance protocols. Current guidelines recommend surveillance for up to 3 years post-treatment, but factors, such as patient demographics and tumor characteristics, may warrant extended monitoring periods. Emerging biomarkers, such as Merkel cell polyomavirus status and circulating tumor DNA, show promise in predicting and monitoring recurrences, but their utility in late recurrence detection requires further investigation.

摘要

关键临床信息

默克尔细胞癌(MCC)由于复发率各异且随访方案不确定,在监测方面存在挑战,尤其是在晚期复发病例中。这些病例需要超越传统时间线的个性化监测策略,例如临床和分子因素,以优化患者预后。

摘要

默克尔细胞癌(MCC)是一种罕见的侵袭性皮肤癌,具有神经内分泌分化,初始治疗后易复发。MCC患者的监测策略缺乏特异性,监测持续时间仍不确定,这给确定合适的随访间隔带来了挑战。因此,我们报告一名94岁女性,21年前左鼻壁有IA期MCC病史,现左小指出现一个圆顶状糜烂结节。活检显示具有特征性的MCC特征,CD56、突触素和CK20免疫组化呈阳性(核周点状)。患者选择不进行进一步的影像学检查或淋巴结活检,接受了莫氏显微外科手术。迄今为止,尚无先前部位复发或新原发性病变发生的证据。该病例强调了尽管无病间隔时间长,但仍需持续监测。它也是文献中初诊MCC后潜伏期/无复发间隔时间最长的病例。虽然大多数复发发生在诊断后的头几年内,但我们的病例突出了晚期复发的特殊性,并促使重新评估监测方案。目前的指南建议治疗后监测3年,但患者人口统计学和肿瘤特征等因素可能需要延长监测期。新兴的生物标志物,如默克尔细胞多瘤病毒状态和循环肿瘤DNA,在预测和监测复发方面显示出前景,但其在晚期复发检测中的效用需要进一步研究。

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本文引用的文献

1
Case report: Metastatic Merkel cell carcinoma presenting seven years after loco-regional disease resection of primary tumor with interval in-transit and nodal metastases.病例报告:原发性肿瘤局部区域疾病切除术后七年出现转移性默克尔细胞癌,期间伴有皮下转移和淋巴结转移。
Front Oncol. 2023 Jul 5;13:1217816. doi: 10.3389/fonc.2023.1217816. eCollection 2023.
2
Trends in incidence, treatment and survival of Merkel cell carcinoma in England 2004-2018: a cohort study.2004-2018 年英国 Merkel 细胞癌发病、治疗和生存趋势:一项队列研究。
Br J Dermatol. 2023 Feb 10;188(2):228-236. doi: 10.1093/bjd/ljac044.
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Society for Immunotherapy of Cancer (SITC) clinical practice guideline on immunotherapy for the treatment of nonmelanoma skin cancer.
癌症免疫治疗学会(SITC)临床实践指南:免疫疗法治疗非黑色素瘤皮肤癌。
J Immunother Cancer. 2022 Jul;10(7). doi: 10.1136/jitc-2021-004434.
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Circulating tumor DNA as a predictive biomarker in Merkel cell carcinoma.循环肿瘤DNA作为默克尔细胞癌的预测生物标志物
J Am Acad Dermatol. 2022 Nov;87(5):1209-1211. doi: 10.1016/j.jaad.2022.03.020. Epub 2022 Mar 19.
5
Recurrence and Mortality Risk of Merkel Cell Carcinoma by Cancer Stage and Time From Diagnosis. Merkel 细胞癌的复发和死亡率与癌症分期及诊断后时间的关系。
JAMA Dermatol. 2022 Apr 1;158(4):382-389. doi: 10.1001/jamadermatol.2021.6096.
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Merkel Cell Polyomavirus Antibody Titer Predicts Recurrence-Free Survival. Merkel 细胞多瘤病毒抗体滴度可预测无复发生存。
Ann Surg Oncol. 2022 Mar;29(3):1620-1626. doi: 10.1245/s10434-021-11008-8. Epub 2021 Dec 1.
7
Merkel cell carcinoma can be indolent: A case with 7 locoregional recurrences over 15 years highlights the importance of patient-tailored management.默克尔细胞癌可能呈惰性:一例15年间出现7次局部区域复发的病例凸显了个体化治疗的重要性。
JAAD Case Rep. 2021 Aug 20;16:58-61. doi: 10.1016/j.jdcr.2021.08.009. eCollection 2021 Oct.
8
Virus-positive Merkel Cell Carcinoma Is an Independent Prognostic Group with Distinct Predictive Biomarkers.病毒阳性 Merkel 细胞癌是一个独立的预后分组,具有独特的预测性生物标志物。
Clin Cancer Res. 2021 May 1;27(9):2494-2504. doi: 10.1158/1078-0432.CCR-20-0864. Epub 2021 Feb 5.
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Merkel cell carcinoma: Current US incidence and projected increases based on changing demographics.默克尔细胞癌:基于人口结构变化的美国当前发病率和预计增长。
J Am Acad Dermatol. 2018 Mar;78(3):457-463.e2. doi: 10.1016/j.jaad.2017.10.028. Epub 2017 Nov 2.
10
Multiple Merkel cell carcinomas: Late metastasis or multiple primary tumors? A molecular study.多发性默克尔细胞癌:晚期转移还是多原发性肿瘤?一项分子研究。
JAAD Case Rep. 2017 Mar 20;3(2):131-134. doi: 10.1016/j.jdcr.2017.01.011. eCollection 2017 Mar.