2020 Edition
| Editors: Maria Rosaria Raspollini, Antonio Lopez-Beltran

Clear Cell Papillary Renal Cell Carcinoma

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-030-41894-6_4791


Clear cell tubulopapillary renal cell carcinoma


Indolent renal cell tumor made up of bland clear cells arranged in tubules and papillae with often linear nuclear alignment away from the basement membrane (Massari et al. 2018).

Clinical Features

  • Incidence

    This neoplasm accounts for 1–4% of renal tumors. Most tumors are incidentally detected as sporadic or arising in end-stage renal disease and von Hippel-Lindau syndrome (Tickoo et al. 2006; Gobbo et al. 2008).

  • Age

    There is no age predilection.

  • Sex

    There is no gender predilection.

  • Site

    There is no site predilection.

  • Treatment

    Partial nephrectomy and less frequently, radical nephrectomy is the standard treatment.

  • Outcome

    To date, neither recurrence nor metastasis has been reported (Massari et al. 2018).


Tumors are often small (usually pT1a), grayish, well-circumscribed and encapsulated with common cystic change (Fig. 1a).
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References and Further Reading

  1. Gobbo, S., Eble, J. N., Grignon, D. J., et al. (2008). Clear cell papillary renal cell carcinoma: a distinct histopathologic and molecular genetic entity. The American Journal of Surgical Pathology, 32, 1239–1245.PubMedCrossRefGoogle Scholar
  2. Martignoni, G., Brunelli, M., Segala, D., et al. (2017). Validation of 34betaE12 immunoexpression in clear cell papillary renal cell carcinoma as a sensitive biomarker. Pathology, 49, 10–18.PubMedCrossRefGoogle Scholar
  3. Massari, F., Ciccarese, C., Hes, O., et al. (2018). The Tumor Entity Denominated “clear cell-papillary renal cell carcinoma” According to the WHO 2016 new Classification, have the Clinical Characters of a Renal Cell Adenoma as does Harbor a Benign Outcome. Pathology Oncology Research, 24, 447–456.PubMedCrossRefGoogle Scholar
  4. Rohan, S. M., Xiao, Y., Liang, Y., et al. (2011). Clear-cell papillary renal cell carcinoma: molecular and immunohistochemical analysis with emphasis on the von Hippel-Lindau gene and hypoxia-inducible factor pathway-related proteins. Modern Pathology, 24, 1207–1220.PubMedCrossRefGoogle Scholar
  5. Tickoo, S. K., dePeralta-Venturina, M. N., Harik, L. R., et al. (2006). Spectrum of epithelial neoplasms in end-stage renal disease: an experience from 66 tumor-bearing kidneys with emphasis on histologic patterns distinct from those in sporadic adult renal neoplasia. The American Journal of Surgical Pathology, 30, 141–153.PubMedPubMedCentralCrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Diagnostic and Public Health, Section of PathologyUniversity of VeronaVeronaItaly
  2. 2.Department of PathologyPederzoli HospitalPeschiera del GardaItaly