Pathological and molecular biological aspects of the renal epithelial neoplasms, up-to-date

Renal neoplasms are not necessarily high in frequency, but they are characteristic in their heterogeneity and occasional association with systemic familial tumor syndromes and phacomatoses (e.g. clear cell renal cell carcinoma and von Hippel-Lindau disease, Wilms tumor and aniridia, genitourinary malformation and mental retardation (so-called, WAGR syndrome), and angiomyolipoma and tuberous sclerosis). Physicians and pathologists should take note of these syndromes and associated renal neoplasms because they have provided important clues to elucidate the mechanism of tumorigenesis concerning cancer-suppressor genes. This review aims to present recent classification of renal parenchymal neoplasms based on their molecular biological characteristics, and future problems yet to be clarified.