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Tubulocystic Renal Cell Carcinoma

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  • First Online:
Encyclopedia of Pathology

Part of the book series: Encyclopedia of Pathology ((EP))

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Definition

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.

Macroscopy

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.

Microscopy

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

  • 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.

    Article  Google Scholar 

  • 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.

    Article  CAS  Google Scholar 

  • 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.

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Diagnostic and Public Health, Section of Pathology, University of Verona, Verona, Italy

    Anna Caliò & Guido Martignoni

  2. Department of Pathology, Pederzoli Hospital, Peschiera del Garda, Italy

    Diego Segala & Guido Martignoni

Authors
  1. Anna Caliò
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  2. Diego Segala
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  3. Guido Martignoni
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Corresponding author

Correspondence to Guido Martignoni .

Editor information

Editors and Affiliations

  1. NIJMEGEN, The Netherlands

    J.H.J.M. van Krieken

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Caliò, A., Segala, D., Martignoni, G. (2019). Tubulocystic Renal Cell Carcinoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_4962-1

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  • DOI: https://doi.org/10.1007/978-3-319-28845-1_4962-1

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-28845-1

  • Online ISBN: 978-3-319-28845-1

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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