探讨肾上腺皮质癌(ACC)和恶性嗜铬细胞瘤(MPCC)的临床病理特征及预后。
我们在日本使用了以医院为基础的癌症登记数据来提取经组织学证实的肾上腺恶性肿瘤病例,诊断,最初从2012-2015年开始治疗。对于生存分析,我们使用来自2008-2009年队列的数据,通过Kaplan-Meier方法估计5年总生存期(OS).
在2012-2015年队列中共发现989例肾上腺恶性肿瘤。最常见的组织学是ACC(26.4%),弥漫性大B细胞淋巴瘤(DLBCL;25.4%),神经母细胞瘤(22.2%),和MPCC(11.9%)。虽然大多数ACC和MPCC患者都在60多岁,在70岁以上的队列中,DLBCL患者占肾上腺恶性肿瘤的61.5%。在有临床分期数据的ACC患者中,46.3%的患者为IV期。尽管手术是所有阶段的主要策略,年轻患者倾向于接受联合治疗,包括手术和化疗或激素治疗。在2008-2009年队列中,ACC(n=49)和MPCC(n=23)患者的5年OS率分别为56.2%和86.4%,而未手术的ACC患者的1年和2年OS率分别为25.0%和12.5%.
在日本,DLBCL占老年患者肾上腺恶性肿瘤的大多数。尽管提前分期,ACC患者以手术为主,预后不理想。这些流行病学数据可能有助于考虑初始管理策略。
To identify the clinicopathological features of adrenal malignancies and analyze the prognoses of patients with adrenal cortical carcinoma (ACC) and malignant pheochromocytoma (MPCC).
We used a hospital-based cancer registry data in Japan to extract cases of adrenal malignancies that were histologically confirmed, diagnosed, and initially treated from 2012-2015. For survival analysis, we used data from the 2008-2009 cohort to estimate 5-year overall survival (OS) by the Kaplan-Meier method.
A total of 989 adrenal malignancies were identified in the 2012-2015 cohort. The most common histologies were ACC (26.4%), diffuse large B-cell lymphoma (DLBCL; 25.4%), neuroblastoma (22.2%), and MPCC (11.9%). While most ACC and MPCC patients were in their 60s, DLBCL patients accounted for 61.5% of adrenal malignancies in the over-70 cohort. Among ACC patients with clinical staging data, 46.3% of patients were stage IV. Although surgery was a chief strategy for all stages, younger patients tended to receive combination therapy, including surgery and chemotherapy or hormone therapy. In the 2008-2009 cohort, the 5-year OS rates of ACC (n = 49) and MPCC (n = 23) patients were 56.2% and 86.4% while ACC patients without surgery had 1- and 2-year OS rates of 25.0% and 12.5%.
In Japan, DLBCL accounted for the majority of adrenal malignancies in older patients. Despite advanced staging, ACC patients were mainly treated with surgery and their prognosis was not satisfactory. Such epidemiological data may be useful in considering initial management strategies.