Volume 81, Issue 1 pp. 15-31
Review

The pathological and molecular genetic landscape of the hereditary renal cancer predisposition syndromes

Khaleel I Al-Obaidy

Khaleel I Al-Obaidy

Department of Pathology, Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA

These authors contributed equally to this paper.

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Zainab I Alruwaii

Zainab I Alruwaii

Department of Pathology, Dammam Regional Laboratory and Blood Bank, Dammam, Saudi Arabia

These authors contributed equally to this paper.

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Sean R Williamson

Sean R Williamson

Department of Pathology, Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, OH, USA

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Liang Cheng

Corresponding Author

Liang Cheng

Department of Pathology, Indiana University School of Medicine, Indianapolis, IN, USA

Department of Urology, Indiana University School of Medicine, Indianapolis, IN, USA

Address for correspondence: L Cheng MD, Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, 350 West 11th Street, Room 4010, Indianapolis, Indiana 46202, USA. e-mail: [email protected]Search for more papers by this author
First published: 22 March 2022
Citations: 1

Abstract

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.

Graphical Abstract

In this review, we summarize the current understanding of the genetic renal neoplasia syndromes, the clinical and pathologic presentations, their molecular pathogenesis, the advances in therapeutic implications and targeted therapy.

Conflicts of interest

The authors have no conflicts of interest to declare.

Data availability statement

Data sharing is not applicable to this article, as no new data were created or analysed in this study.

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