背景:乳头状肾细胞癌(pRCC)是肾细胞癌(RCC)的第二常见组织学,占病例的10-15%。传统上,pRCC分为1型和2型,尽管这种划分目前被认为是生存的预后因素。我们的目的是在50年内调查整个国家患者队列中pRCC亚型的流行病学和生存率。
方法:一项基于人群的回顾性研究,包括1971-2020年冰岛连续RCC病例。对RCC的组织学分类进行了比较,重点是pRCC亚型(1型与2)用于结果估计。年龄标准化后,每隔5年分析RCC发病率的变化。结果分析采用Kaplan-Meier法和Cox回归分析。
结果:共确认1.725例,74.4%,2.1%和9.2%有透明细胞(ccRCC),发色(chRCC),和pRCC,分别。pRCC的年龄标准化发病率(ASI)男性为1.97/100.000,女性为0.5/100.000,pRCC的比例在研究的第一个和最后一个间隔之间从3.7%增加到11.5%(p<0.001)。男性和女性的年龄标准化癌症特异性死亡率(ASCSM)为0.6/100.000和0.19/100.000。分别。1型pRCC的ASI年平均增长率为3.6%,但2型pRCC的ASI和两种亚型的ASCSM没有显著变化.1型pRCC的男女比例为4.4,2型为2.3。1型和2型的平均肿瘤大小分别为58.8和73.7mm,分别。在1型pRCC中发现了8.7%的转移,与30.0%的2型pRCC患者相比(p<0.001)。估计的5年癌症特异性生存率(CSS)为94.4%,80.7%,chRCC为69.3%,pRCC和ccRCC,分别(p<0.001)。对于pRCC子类型,与类型2相比,类型1与更好的5年CSS相关(86.3%vs.66.0%,p<0.001),尽管在调整癌症分期和分级后,这种差异并不显著。
结论:pRCC组织学在冰岛比在其他国家稍不常见。男性被诊断为pRCC的可能性是男性的三倍以上,与其他RCC组织学相比。未发现pRCC亚型是生存率较差的独立危险因素。正如世界卫生组织最新的泌尿系肿瘤分类所建议的那样,分级和TNM分期似乎是评估pRCC患者生存的最重要因素。
BACKGROUND: Papillary renal cell carcinoma (pRCC) is the second most common histology of renal cell carcinoma (RCC), accounting for 10-15% of cases. Traditionally, pRCC is divided into type 1 and type 2, although this division is currently debated as a prognostic factor of survival. Our aim was to investigate the epidemiology and survival of the pRCC subtypes in a whole nation cohort of patients during a 50-year period.
METHODS: A Population based retrospective
study including consecutive cases of RCC in Iceland from 1971-2020. Comparisons were made between histological classifications of RCC, with emphasis on pRCC subtypes (type 1 vs. 2) for outcome estimation. Changes in RCC incidence were analyzed in 5-year intervals after age standardization. The Kaplan-Meier method and Cox regression were used for outcome analysis.
RESULTS: A total of 1.725 cases were identified, with 74.4%, 2.1% and 9.2% having clear cell (ccRCC), chromophobe (chRCC), and pRCC, respectively. The age standardized incidence (ASI) of pRCC was 1.97/100.000 for males and 0.5/100.000 for females, and the proportion of pRCC increased from 3.7% to 11.5% between the first and last intervals of the
study (p < 0.001). Age standardized cancer specific mortality (ASCSM) of pRCC was 0.6/100.000 and 0.19/100.000 for males and females, respectively. The annual average increase in ASI was 3.6% for type 1 pRCC, but the ASI for type 2 pRCC and ASCSM for both subtypes did not change significantly. Male to female ratio was 4.4 for type 1 pRCC and 2.3 for type 2. The average tumor size for type 1 and 2 was 58.8 and 73.7 mm, respectively. Metastasis at diagnosis was found in 8.7% in the type 1 pRCC, compared to 30.0% of patients with type 2 pRCC (p < 0.001). Estimated 5-year cancer-specific survival (CSS) were 94.4%, 80.7%, and 69.3% for chRCC, pRCC and ccRCC, respectively (p < 0.001). For the pRCC subtypes, type 1 was associated with better 5-year CSS than type 2 (86.3% vs. 66.0%, p < 0.001), although this difference was not significant after adjusting for cancer stage and grading.
CONCLUSIONS: pRCC histology was slightly less common in Iceland than in other countries. Males are more than three times more likely to be diagnosed with pRCC, compared to other RCC histologies. The subtype of pRCC was not found to be an independent risk factor for worse survival, and as suggested by the most recent WHO Classification of Urinary Tumors, grade and TNM-stage seem to be the most important factors for estimation of survival for pRCC patients.