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Papillary Renal Cell Carcinoma (PRCC)

An Update

Akhtar, Mohammed, MD, FCAP, FRCPA, FRCPath*; Al-Bozom, Issam A., MD*; Al Hussain, Turki, MD

Advances in Anatomic Pathology: March 2019 - Volume 26 - Issue 2 - p 124–132
doi: 10.1097/PAP.0000000000000220
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Papillary renal cell carcinoma (PRCC) is the second most common type of renal carcinoma following clear cell renal cell carcinoma. Papillary renal cell carcinoma is usually divided histologically into 2 types namely, type 1 and type 2. This classification, however, is unsatisfactory as many of papillary carcinoma are unclassifiable by the existing criteria. In recent years there has been a remarkable progress in our understanding of the molecular basis of PRCC. These studies have revealed that type 2 PRCCs represent a heterogenous group which may be subdivided into additional subtypes based on the genetic and molecular make up of these tumors and reflecting different clinical course and prognosis. Some of the molecular features such a hypermethylation of CPG islands in the promotor regions of genes and over expression of the antioxidant pathways within tumor cells have been recognized as markers of poor prognosis. Targeted therapies for papillary carcinoma in the past have been unsuccessful because of lack of clear understanding of the molecular basis of these tumors. It is hoped that recent progress in our understanding of the pathogenesis of various subtypes of PRCC, effective targeted therapies will eventually emerge in due course.

*Department of Laboratory Medicine and Pathology, Hamad Medical Corporation, Doha, Qatar

Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia

The authors have no funding or conflicts of interest to disclose.

All figures can be viewed online in color at www.anatomicpathology.com.

Reprints: Mohammed Akhtar, MD, FCAP, FRCPA, FRCPath, Department of Laboratory Medicine and Pathology, Hamad Medical Corporation, P.O. Box 3050, Doha, Qatar (e-mail: makhtar@hamad.qa).

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