School of Medicine, Western Sydney University, Sydney, New South Wales, Australia.
Macarthur Cancer Therapy Centre, Campbelltown Hospital, Sydney, New South Wales, Australia.
Intern Med J. 2024 Mar;54(3):414-420. doi: 10.1111/imj.16281. Epub 2023 Nov 27.
Extrapulmonary small cell carcinomas (EPSCCs) are rare cancers, comprising 0.1-0.4% of all cancers. The scarcity of EPSCC studies has led current treatment strategies to be extrapolated from small cell lung cancer (SCLC), justified by analogous histological and clinical features.
We conducted a retrospective cohort study comparing the outcomes of extensive-stage (ES) SCLC and EPSCC.
Patients diagnosed with ES SCLC or EPSCC between 2010 and 2020 from four hospitals in Sydney were identified. Patients who received active treatment and best supportive care were included. The primary endpoint was overall survival (OS), and secondary endpoints were progression-free survival (PFS) and overall response rates (ORRs).
Three hundred and eighty-four patients were included (43 EPSCC vs. 340 SCLC). EPSCC were of genitourinary (n = 15), unknown primary (n = 13) and gastrointestinal (n = 12) origin. Treatment modalities for EPSCC compared to SCLC included palliative chemotherapy (56% vs 73%), palliative radiotherapy (47% vs 59%) and consolidation chest radiotherapy (10% of SCLC). Overall, median OS was 6.4 versus 7 months for EPSCC versus SCLC respectively, but highest in prostate EPSCC (25.6 months). Of those who received chemotherapy (22 EPSCC vs 233 SCLC), median OS was 10.4 versus 8.4 months (HR OS 0.81, 95% confidence interval (CI): 0.5-1.31, P = 0.38); PFS was 5.4 versus 5.5 months (HR PFS 0.93, 95% CI: 0.58-1.46, P = 0.74) and ORR were 73% versus 68%.
EPSCC and SCLC appeared to have comparable OS and treatment outcomes. However, the wide range of OS in EPSCC highlights the need for an improved understanding of its genomics to explore alternative therapeutics.
肺外小细胞癌（EPSCC）是一种罕见的癌症，占所有癌症的 0.1-0.4%。由于 EPSCC 研究较少，目前的治疗策略是从小细胞肺癌（SCLC）中推断出来的，这是基于类似的组织学和临床特征。
我们进行了一项回顾性队列研究，比较了广泛期（ES）SCLC 和 EPSCC 的结果。
从悉尼的四家医院确定了 2010 年至 2020 年间诊断为 ES SCLC 或 EPSCC 的患者。纳入接受积极治疗和最佳支持治疗的患者。主要终点是总生存期（OS），次要终点是无进展生存期（PFS）和总缓解率（ORR）。
共纳入 384 例患者（43 例 EPSCC 与 340 例 SCLC）。EPSCC 来源于泌尿生殖系统（n=15）、未知原发灶（n=13）和胃肠道（n=12）。与 SCLC 相比，EPSCC 的治疗方式包括姑息性化疗（56%比 73%）、姑息性放疗（47%比 59%）和巩固胸部放疗（SCLC 的 10%）。总的来说，EPSCC 和 SCLC 的中位 OS 分别为 6.4 个月和 7 个月，但前列腺 EPSCC 最高（25.6 个月）。接受化疗的患者中（22 例 EPSCC 与 233 例 SCLC），中位 OS 分别为 10.4 个月和 8.4 个月（OS 风险比 0.81，95%置信区间（CI）：0.5-1.31，P=0.38）；PFS 分别为 5.4 个月和 5.5 个月（PFS 风险比 0.93，95%CI：0.58-1.46，P=0.74），ORR 分别为 73%和 68%。
EPSCC 和 SCLC 似乎具有相似的 OS 和治疗结果。然而，EPSCC 的 OS 范围广泛，这突出表明需要更好地了解其基因组学，以探索替代治疗方法。
