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

Tubulocystic Renal Cell Carcinoma

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-030-41894-6_4962


Tubulocystic carcinoma is an uncommon multicystic renal cell tumor.

Clinical Features

  • Incidence

    The tumor comprises less than 1% of all renal carcinomas.

  • Age

    Tubulocystic carcinoma occurs most commonly in the fifth and sixth decades of life.

  • Sex

    There is a male predominance.

  • Site

    There is no site predilection.

  • Treatment

    Radical nephrectomy is generally recommended, but partial nephrectomy may be performed for small tumors located in the superficial renal cortex.

  • Outcome

    They usually display an indolent behavior with a few cases with metastasis or recurrence.


The tumors are solitary well-circumscribed multicystic renal mass. They are frequently 4 cm or less and composed of multiple small- to intermediate-sized cysts and have a white and sponge-like cut surface.


The cysts above described are lined by a single layer of flattened, cuboidal, and hobnailed eosinophilic renal cells with enlarged nuclei with prominent (ISUP/WHO 2016 grade 3) nucleoli (Fig. 1)....
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References and Further Reading

  1. Amin, M. B., MacLennan, G. T., Gupta, R., et al. (2009). Tubulocystic carcinoma of the kidney: Clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma. The American Journal of Surgical Pathology, 33, 384–392.CrossRefGoogle Scholar
  2. Sarungbam, J., Mehra, R., Tomlins, S. A., et al. (2019). Tubulocystic renal cell carcinoma: A distinct clinicopathologic entity with a characteristic genomic profile. Mod Pathol, 32, 701–709.CrossRefGoogle Scholar
  3. Yang, X. J., Zhou, M., Hes, O., et al. (2008). Tubulocystic carcinoma of the kidney: Clinicopathologic and molecular characterization. The American Journal of Surgical Pathology, 32, 177–187.CrossRefGoogle 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