Henry N, Rouach Y, Nguyen K, Verkarre V, Mejean A, Timsit M-O
Université Paris Descartes, 12, rue de l'Ecole de Médecine, 75270 Paris cedex 06, France.
Prog Urol. 2010 Jun;20(6):393-401. doi: 10.1016/j.purol.2010.02.003. Epub 2010 Mar 25.
We reviewed papillary renal cell carcinoma (PRCC) epidemiology, radiological and clinical presentations, and specific features of morphological subtypes focusing on genetic defects, risk of local and metastatic recurrence and frequency of multifocality.
The MEDLINE database of the US National Library of Medicine was searched for pertinent studies.
According to multivariate analyses, PRCC histology was not retained as a prognostic factor. Reported rates of multifocality in PRCC are 22 to 41% but distinction between histological subtype or hereditary forms are barely detailed. Multifocality frequency is independent of size, stage or grade and is not associated with ipsilateral or controlateral recurrence or death from RCC. Thus, PRCC multifocality is not an argument against nephron-sparing surgery. Antiangionenic therapies are being evaluated for the Metastatic PRCC.
According to the literature, specific prognostic features of PRCC remain controversial due to the lack of distinction between different PRCC subtypes. Genomic and cytogenetic characterizations have been used to establish an evolving classification of PRCC subtypes and may be a source of new markers that will eventually enable us to precise prognosis and identify targets for new adjuvant therapies.
我们回顾了乳头状肾细胞癌（PRCC）的流行病学、放射学和临床表现，以及形态学亚型的特定特征，重点关注基因缺陷、局部和转移复发风险以及多灶性频率。
检索美国国立医学图书馆的MEDLINE数据库以查找相关研究。
根据多变量分析，PRCC组织学未被保留为预后因素。报道的PRCC多灶性发生率为22%至41%，但组织学亚型或遗传形式之间的区别几乎没有详细说明。多灶性频率与大小、分期或分级无关，且与同侧或对侧复发或肾癌死亡无关。因此，PRCC多灶性并非反对保留肾单位手术的理由。抗血管生成疗法正在转移性PRCC中进行评估。
根据文献，由于不同PRCC亚型之间缺乏区分，PRCC的特定预后特征仍存在争议。基因组和细胞遗传学特征已被用于建立PRCC亚型的不断发展的分类，并且可能是新标志物的来源，最终将使我们能够精确预后并确定新辅助治疗的靶点。
