Mindy X Wang, Mahmoud Diab, Albert R Klekers, Khaled M Elsayes
Hereditary renal cancer syndromes are rare, yet contribute to early-onset renal cell carcinoma (RCC) and require vigilant imaging surveillance. These syndromes, including Von Hippel-Lindau, Birt-Hogg-Dubé, hereditary papillary RCC, are linked to germline mutations affecting tumor suppressor pathways. Imaging techniques, such as MR imaging and computed tomography, play a crucial role in early diagnosis, characterization, and surveillance of hereditary RCC. A multidisciplinary approach is essential for individualized screening and management strategies. This review highlights the genetic associations, imaging features, and most up-to-date recommendations for screening and surveillance of hereditary RCC syndromes.