Division of Dermatology, Department of Medicine, University of Washington, Seattle.
Division of Dermatology, Department of Medicine, Washington University in St Louis, St Louis, Missouri.
JAMA Dermatol. 2022 Apr 1;158(4):382-389. doi: 10.1001/jamadermatol.2021.6096.
Merkel cell carcinoma (MCC) often behaves aggressively; however, disease-recurrence data are not captured in national databases, and it is unclear what proportion of patients with MCC experience a recurrence (estimates vary from 27%-77%). Stage-specific recurrence data that includes time from diagnosis would provide more precise prognostic information and contribute to risk-appropriate clinical surveillance.
To estimate risk of stage-specific MCC recurrence and mortality over time since diagnosis.
DESIGN, SETTING, AND PARTICIPANTS: This prospective cohort study included 618 patients with MCC who were prospectively enrolled in a Seattle-based data repository between 2003 and 2019. Of these patients, 223 experienced a recurrence of MCC. Data analysis was performed July 2019 to November 2021.
Stage-specific recurrence and survival, as well as cumulative incidence and Kaplan-Meier analyses.
Among the 618 patients included in the analysis (median [range] age, 69 [11-98] years; 227 [37%] female), the 5-year recurrence rate for MCC was 40%. Risk of recurrence in the first year was high (11% for patients with pathologic stage I, 33% for pathologic stage IIA/IIB, 30% for pathologic stage IIIA, 45% for pathologic stage IIIB, and 58% for pathologic stage IV), with 95% of recurrences occurring within the first 3 years. Median follow-up among living patients was 4.3 years. Beyond stage, 4 factors were associated with increased recurrence risk in univariable analyses: immunosuppression (hazard ratio [HR], 2.4; 95% CI, 1.7-3.3; P < .001), male sex (HR, 1.9; 95% CI, 1.4-2.5; P < .001), known primary lesion among patients with clinically detectable nodal disease (HR, 2.3; 95% CI, 1.4-4.0; P = .001), and older age (HR, 1.1; 95% CI, 1.0-1.3; P = .06 for each 10-year increase). Among 187 deaths in the cohort, 121 (65%) were due to MCC. The MCC-specific survival rate was strongly stage dependent (95% at 5 years for patients with pathologic stage I vs 41% for pathologic stage IV). Among patients presenting with stage I to II MCC, a local recurrence (17 arising within/adjacent to the primary tumor scar) did not appreciably diminish survival compared with patients who had no recurrence (85% vs 88% MCC-specific survival at 5 years).
In this cohort study, the MCC recurrence rate (approximately 40%) was notably different than that reported for invasive melanoma (approximately 19%), squamous cell carcinoma (approximately 5%-9%), or basal cell carcinoma (approximately 1%-2%) following definitive therapy. Because more than 90% of MCC recurrences arise within 3 years, it is appropriate to adjust surveillance intensity accordingly. Stage- and time-specific recurrence data can assist in appropriately focusing surveillance resources on patients and time intervals in which recurrence risk is highest.
Merkel 细胞癌 (MCC) 通常表现出侵袭性；然而，国家数据库中并未捕获疾病复发数据，并且尚不清楚有多少 MCC 患者会经历复发（估计范围为 27%-77%）。包括从诊断到复发时间的特定于疾病分期的复发数据将提供更精确的预后信息，并有助于进行适当的临床监测。
估计自诊断以来特定于疾病分期的 MCC 复发和死亡率随时间的变化情况。
设计、设置和参与者： 这项前瞻性队列研究纳入了 2003 年至 2019 年间在西雅图的一个数据存储库中前瞻性登记的 618 例 MCC 患者。其中 223 例患者出现 MCC 复发。数据分析于 2019 年 7 月至 2021 年 11 月进行。
特定于疾病分期的复发和生存情况，以及累积发病率和 Kaplan-Meier 分析。
在纳入分析的 618 例患者中（中位[范围]年龄，69 [11-98] 岁；227 [37%] 为女性），MCC 的 5 年复发率为 40%。第一年的复发风险较高（病理分期 I 患者为 11%，病理分期 IIA/IIB 为 33%，病理分期 IIIA 为 30%，病理分期 IIIB 为 45%，病理分期 IV 为 58%），95%的复发发生在头 3 年内。生存患者的中位随访时间为 4.3 年。除疾病分期外，4 个因素在单变量分析中与复发风险增加相关：免疫抑制（风险比 [HR]，2.4；95%置信区间 [CI]，1.7-3.3；P<0.001）、男性（HR，1.9；95% CI，1.4-2.5；P<0.001）、已知原发性病变（HR，2.3；95% CI，1.4-4.0；P=0.001）和年龄较大（HR，1.1；95% CI，每增加 10 岁增加 1.0-1.3；P=0.06）。在队列中 187 例死亡中，121 例（65%）死于 MCC。MCC 特异性生存率与疾病分期密切相关（病理分期 I 患者的 5 年生存率为 95%，而病理分期 IV 患者的生存率为 41%）。在表现为 I 期至 II 期 MCC 的患者中，局部复发（17 例发生在原发性肿瘤瘢痕内/附近）与无复发患者的生存情况相比并没有明显降低（5 年 MCC 特异性生存率分别为 85%和 88%）。
在这项队列研究中，MCC 的复发率（约 40%）与侵袭性黑色素瘤（约 19%）、鳞状细胞癌（约 5%-9%）或基底细胞癌（约 1%-2%）在接受确定性治疗后的复发率显著不同。由于超过 90%的 MCC 复发发生在 3 年内，因此可以相应地调整监测强度。疾病分期和时间特异性复发数据有助于适当地将监测资源集中在复发风险最高的患者和时间段上。
