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Misdiagnosis of clear cell renal cell carcinoma

Nature Reviews Urology volume 8, pages 321–333 (2011)Cite this article

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Abstract

Clear cell renal cell carcinoma (RCC) represents the most common histological subtype of malignant kidney tumors. Based on symptoms alone, clear cell RCC is indistinguishable from other histological classes of RCC unless the tumor is present in the context of an RCC syndrome. Histopathological examination is, therefore, important to accurately identify clear cell RCC. Clear cell RCCs have characteristic morphological criteria; these tumors can be easily identified upon typical presentation, but diagnosis can be challenging when tumor cell pattern is unusual or when availability of tissue samples is limited. In this Review, the clinical, radiological and pathological characteristics of clear cell RCCs are described, as well as the potential tumors that can be confused with clear cell RCC and need to be considered in the differential diagnoses. Finally, the importance of an accurate diagnosis is highlighted in the context of the increasing use of preoperative tissue sampling and the prevalence of clear cell tumors associated with hereditary syndromes, which could have different therapeutic and prognostic implications for patients and their families.

Key Points

  • Renal cell carcinoma (RCC) is a type of renal tumor that encompasses a group of different morphological and genetic diseases

  • RCC can occur as sporadic cases or as part of hereditary syndromes

  • Morphological differences between subtypes are supported by underlying genetic changes

  • Clinically and radiologically, histotypes can be difficult to differentiate, especially for clear cell RCC

  • Misdiagnosis or misclassification of RCC can be minimized by extensive sampling of tumors

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Figure 1: Macroscopic appearance of a typical clear cell RCC.
Figure 2: Microscopic features of clear cell (conventional) RCCs.
Figure 3: Architectural configurations in clear cell RCC.
Figure 4: Fuhrman nuclear grade classification of renal tumors.
Figure 5: Ultrastructural characteristics of clear cell RCC.

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Acknowledgements

This research was supported in part by the Intramural Research Program of the National Cancer Institute, NIH. The content of this publication does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the US Government. C. P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape, LLC-accredited continuing medical education activity associated with this article.

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  1. Translational Surgical Pathology section, Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 10 Center Drive, Building 10, MSC Room 2B44, Bethesda, 20892, MD, USA

    Vladimir A. Valera & Maria J. Merino

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Correspondence to Maria J. Merino.

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Valera, V., Merino, M. Misdiagnosis of clear cell renal cell carcinoma. Nat Rev Urol 8, 321–333 (2011). https://doi.org/10.1038/nrurol.2011.64

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