Massari Francesco, Ciccarese Chiara, Hes Ondrej, Michal Michal, Caliò Anna, Fiorentino Michelangelo, Giunchi Francesca, D'Amuri Alessandro, Sanguedolce Francesca, Sabbatini Roberto, Guida Annalisa, Ardizzoni Andrea, Porta Camillo, Iacovelli Roberto, Tortora Giampaolo, Cima Luca, Ortega Cinzia, Lapini Alberto, Martignoni Guido, Brunelli Matteo
Division of Oncology, S.Orsola-Malpighi Hospital, Bologna, Italy.
Division of Oncology, AOUI, University of Verona, Verona, Italy.
Pathol Oncol Res. 2018 Jul;24(3):447-456. doi: 10.1007/s12253-017-0271-x. Epub 2017 Jul 10.
The new WHO 2016 classification of renal neoplasia encounters the new entity called "clear cell papillary renal cell carcinoma" (ccpRCC). The ccpRCC has been long included as a subtype of clear cell RCC histotype and it actually ranges from 2 to 9% in different routinely available cohort of renal carcinomas. Of important note, ccpRCC does not show any recurrences or metastases or lymph-node invasion and the outcome is always good. We reviewed twenty-four publications with available follow-up for patients (no. 362) affected by clear cell papillary RCCs/renal adenomatoid tumours and notably ccpRCC harbors an indolent clinical behavior after a mean of 38 months (3,5 years) of follow-up. This paper reviews the histological, molecular and clinical features characterizing ccpRCC, with the goal of focusing the knowledge of the benign fashion of this new tumour entity, supporting the idea of a new renal cell adenoma recruited morphologically from ex conventional clear cell RCC tumours.
世界卫生组织(WHO)2016年新版肾肿瘤分类中出现了一个新的实体,即“透明细胞乳头状肾细胞癌”(ccpRCC)。长期以来,ccpRCC一直被列为透明细胞肾细胞癌组织学类型的一个亚型,在不同的常规肾癌队列中,其实际占比为2%至9%。值得注意的是,ccpRCC未显示任何复发、转移或淋巴结侵犯,预后始终良好。我们回顾了24篇关于透明细胞乳头状肾细胞癌/肾腺瘤样肿瘤患者(共362例)的随访可用文献,值得注意的是,在平均38个月(3.5年)的随访后,ccpRCC具有惰性临床行为。本文回顾了ccpRCC的组织学、分子和临床特征,目的是聚焦对这个新肿瘤实体良性特征的认识,支持从传统透明细胞肾细胞癌肿瘤形态学上重新归类为一种新的肾细胞腺瘤的观点。