Journal
HISTOPATHOLOGY
Volume 81, Issue 1, Pages 15-31Publisher
WILEY
DOI: 10.1111/his.14641
Keywords
differential diagnosis; hereditary renal cancer syndromes; kidney; molecular genetics; personalized medicine; targeted therapy; von Hippel-Lindau disease
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This review provides an overview of the current understanding, clinical and pathological presentations, molecular pathogenesis, advances in therapeutic implications, and targeted therapy of genetic renal neoplasia syndromes.
It is estimated that 5-8% of renal tumours are hereditary in nature, with many inherited as autosomal-dominant. These tumours carry a unique spectrum of pathological and molecular alterations, the knowledge of which has expanded in recent years. Due to this knowledge, many advances in the treatment of these tumours have been achieved. In this review, we summarize the current understanding of the genetic renal neoplasia syndromes, clinical and pathological presentations, molecular pathogenesis, advances in therapeutic implications and targeted therapy.
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