Recognizing Hereditary Renal Cancers Through the Microscope: A Pathology Update

Abstract

A heightened understanding of hereditary renal cancer syndromes and their molecular basis has led to an increased awareness and recognition of these renal neoplasms by pathologists. Because a diagnosis of hereditary renal cell carcinoma has a profound impact on the patient and family members, when and how to raise such a suspicion via pathologic assessment has become an important yet very challenging task. This review discusses key clinicopathologic, immunohistochemical, and genetic characteristics of hereditary renal cancer syndromes, and important differential diagnostic challenges, emphasizing recent pathologic and molecular advances.

Keywords: Birt-Hogg-Dubé; Familial; Hereditary leiomyomatosis renal cell carcinoma; Hereditary papillary; Kidney cancer; Succinate dehydrogenase; Tuberous sclerosis; von Hippel-Lindau.