Encyclopedia of Pathology

Living Edition
| Editors: J.H.J.M. van Krieken

Clear Cell Papillary Renal Cell Carcinoma

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_4791-1

Synonyms

Definition

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).

Macroscopy

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

Microscopy

Tubular, papillary, branched glandular, acinar, and cystic patterns are seen in varying proportions. Neoplastic clear cells are cuboidal with round nuclei and inconspicuous nucleoli (ISUP/WHO grade 1 and 2) arranged in a linear fashion away from the basement membrane. Fibrous and/or smooth muscle stroma in varying amounts may be observed (Fig. 1b).

Immunophenotype

Tumor cells diffusely express cytokeratin7, PAX8, cytokeratin 34βE12, and CAIX (often in cup-like distribution), frequently stain for GATA3; whereas racemase and CD10 are usually negative (Rohan et al. 2011; Martignoni et al. 2017).

Molecular Features

These tumors lack the classic genetic events of clear cell renal cell carcinoma (3p/VHL alteration) and papillary renal cell carcinoma (7 and 17 trisomies) (Rohan et al. 2011).

Differential Diagnosis

The differential diagnosis with clear cell renal cell carcinoma with low nucleolar grade may be extremely difficult. Despite immunophenotype being usually diagnostic, in selected cases, molecular analysis could be necessary to classify the neoplasm. Less frequently, papillary renal cell carcinoma with extensively clear cell changes should be ruled out.
Fig. 1

A small, well-circumscribed, and encapsulated with cystic changes mass without the yellowish features typically seen in clear cell renal cell carcinoma (a). Cuboidal clear cells with inconspicuous nucleoli in a linear fashion away from the basement membrane arranged in a tubular pattern (b)

References

  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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle 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.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

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