Emerging Entities in Renal Tumor Pathology: What Matters

 

The well-known renal tumor types include clear cell renal cell carcinoma (RCC), papillary RCC, chromophobe RCC, and oncocytoma. However, recent advances in immunohistochemistry and molecular techniques have recognized an expanding number of potential entities. For some of these, the significance of “lumping” vs. “splitting” is uncertain. For example, entities now proposed as low-grade oncocytic tumor (LOT) and eosinophilic vacuolated tumor (EVT) have recognizable morphologies, immunohistochemistry, and genetics, but clinical management like oncocytoma/chromophobe RCC is probably reasonable for most patients. At the same time, other diagnoses may have implications for hereditary syndromes, like SDH and FH-deficient RCC, and still other tumors may be particularly aggressive, like TFEB/6p21 amplified RCC. This presentation will address the emerging entities in renal tumor pathology, with emphasis on clinical implications.

Originally published on June 3, 2025

Lecture Presenter

Sean R. Williamson, MD

Sean R. Williamson, MD

Director, Genitourinary Pathology
Cleveland Clinic
Vice Chair, Education, Department of Pathology and Laboratory Medicine
Cleveland Clinic Diagnostics Institute

Sean R. Williamson, MD, is the director of Genitourinary Pathology and vice chair of Education in the Department of Pathology and Laboratory Medicine of the Cleveland Clinic Diagnostics Institute. He is also a professor of Pathology at the Cleveland Clinic Lerner College of Medicine. Originally from Pittsburgh, Pennsylvania, he studied medicine at Temple University School of Medicine in Philadelphia, followed by residency and genitourinary pathology fellowship training at Indiana University School of Medicine, Indianapolis. Prior to joining the Cleveland Clinic, he practiced in the Henry Ford Health System in the Detroit Metro area, where he was selected by the trainees as the Anatomic Pathology Teacher of the Year 4 times. He is currently editor-in-chief of the International Journal of Surgical Pathology. He has contributed to more than 150 peer-reviewed journal articles and is co-author of a textbook Atlas of Genitourinary Pathology: A Pattern-Based Approach. He is a lead author for multiple sections (urinary bladder, kidney, and mesenchymal tumors) in the recent fifth edition of the World Health Organization Classification of tumors of the urinary system and is a coauthor of the AFIP text, fifth series, on tumors of the testis and paratesticular structures. He currently serves on the editorial boards of several journals, including Modern Pathology and Histopathology, and is the recipient of the 2024 Arthur Purdy Stout Society Annual Prize. His research is focused on diagnostic, prognostic, and staging challenges in surgical pathology, with emphasis on recognizing novel tumor histologic features that correlate with underlying molecular events and significance for patient outcome.

Objectives

After this presentation, participants will be able to:

  • Communicate the significance of emerging renal tumor types
  • Apply immunohistochemical markers to diagnose emerging entities in renal tumor pathology

Sponsored by:

University of Utah School of Medicine, Department of Pathology, and ARUP Laboratories