Encyclopedia of Pathology

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

Tubulocystic 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_4962-1


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). The stroma is fibrotic (Yang et al. 2008; Amin et al. 2009).
Fig. 1

A well-circumscribed multicystic renal mass


Neoplastic cells stain for CK7, 34βE12, AMACR, and CD10.

Molecular Features

Tubulocystic carcinoma shows gains of chromosomes 17 and loss of the Y chromosome and chromosome 9 (Sarungbam et al. 2019).

Differential Diagnosis

The differential diagnosis includes cystic nephroma (immunohistochemical expression of estrogen and progesterone receptor), hereditary leiomyomatosis and renal cell carcinoma-associated renal cell carcinoma (FH loss), and papillary renal cell carcinoma (papillary architecture).

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 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