背景:历史上,IV期肾上腺皮质癌(mACC)预后较差,中位总生存期(OS)仅为5个月.根据2012年发表的FIRM-ACT试验,指南现在建议使用依托泊苷进行一线全身治疗。顺铂,多柔比星和米托坦(EDP-M)。EDP-M在荷兰临床实践中对患者生存的影响尚不清楚。
方法:所有mACC患者(2005-2020)的数据均来自荷兰综合癌症组织(IKNL)。使用Kaplan-Meier分析和多变量Cox回归分析评估EDP-M对患者生存的影响,包括临床,治疗和肿瘤特征。
结果:共纳入167例mACC患者。从2014年起确诊的患者,与未接受EDP-M的患者相比,EDP-M(22例(22%))在数字上但无统计学意义的OS改善(11.8vs5.6个月,p=0.525)。对于全身治疗,仅接受米托坦治疗的患者5年OS最好(11.4%,p=0.006),与诊断年份无关。在多变量Cox回归分析中,EPD-M与OS无关;从2014年开始,姑息性肾上腺切除术(HR:0.26,p=<.001)和局部转移治疗(HR:0.35,p=0.001)与更好的OS和原发肿瘤Ki-67指数>20%(HR:2.67,p=0.003)相关。与2014年以来相比,2014年之前诊断的患者的OS明显较差(5年:4.5vs8.4%,操作系统:6.8和8.3个月,p=0.032)。
结论:在过去十年中,荷兰的mACC操作系统有所改善。接受EDP-M并没有显着改善mACC患者的OS。使用多模式治疗,包括姑息性肾上腺切除术,自2014年以来,在适当选择的患者中,米托坦和(寡)转移的局部治疗改善了mACC患者的OS.
BACKGROUND: Historically, stage IV adrenocortical carcinoma (mACC) has a poor prognosis with a median overall survival (OS) of only 5 months. Based on the FIRM-ACT trial published in 2012, guidelines now advise first line systemic treatment with etoposide, cisplatin, doxorubicin and mitotane (EDP-M). The effect of EDP-M on patient survival in clinical practice in the Netherlands is unknown.
METHODS: The data of all patients with mACC (2005-2020) were obtained from the Netherlands comprehensive cancer organization (IKNL). The effect of EDP-M on patient survival was assessed using Kaplan-Meier analysis and multivariate Cox regression analysis including clinical, therapy and tumor characteristics.
RESULTS: In total 167 patients with mACC were included. For patients diagnosed from 2014 onwards, EDP-M (in 22 patients (22%)) lead to a numerically but not statistically significant improved OS compared to those not receiving EDP-M (11.8 vs 5.6 months, p = 0.525). For systemic treatments, patients treated with mitotane only had the best 5-year OS (11.4%, p = 0.006) regardless of year of diagnosis. In multivariate Cox regression analysis EPD-M was not associated with OS; palliative adrenalectomy (HR: 0.26, p = <.001) and local treatment of metastases (HR: 0.35, p = 0.001) were associated with a better OS and a primary tumor Ki-67 index > 20% (HR: 2.67, p = 0.003) with a worse OS from 2014 onwards. Patients diagnosed before 2014 had a significantly poorer OS compared to from 2014 onwards (5-yr: 4.5 vs 8.4%, OS: 6.8 vs 8.3 months, p = 0.032).
CONCLUSIONS: OS for mACC in the Netherlands has improved in the last decade. Receiving EDP-M did not significantly improve OS for patients with mACC. The use of multimodality treatment including palliative adrenalectomy, mitotane and local treatment of (oligo-)metastases in appropriately selected patients has improved the OS for mACC patients since 2014.