Encyclopedia of Pathology

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

Multilocular Cystic Renal Neoplasm of Low Malignant Potential

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_4855-1


This tumor was previously named “multilocular cystic renal cell carcinoma.”


Multicystic tumor with septa containing individual or groups of clear cells with ISUP/WHO 2016 low nucleolar grade (1–2).

Clinical Features

  • Incidence

    This tumor accounts for less than 1% of all renal tumors, usually incidentally discovered.

  • Age

    It occurs in middle-age adults.

  • Sex

    There is no difference between male and female.

  • Site

    There is no site predilection.

  • Treatment

    Partial or radical nephrectomy is the surgical treatment of choice.

  • Outcome

    To date there are no evidence of recurrence or metastasis.


The tumor consists exclusively of variably sized cysts separated by thin septa and filled with clear, serous, or gelatinous fluid (Fig. 1). Solid, grossly discernible tumor mural nodules are incompatible with the diagnosis.


The cysts are lined by a single layer of neoplastic cells with abundant clear cytoplasm and small nuclei without nucleoli (ISUP/WHO 2016 grade 1 or 2). The septa consist of fibrous tissue with calcification or ossification. An important diagnostic feature is the presence within the fibrous septa of small clusters of tumor cells similar to those lining the cysts. Necrosis, vascular invasion, and sarcomatous transformation are incompatible with this diagnosis.


The neoplastic cells are strong reactive to PAX8 and CAIX, similar to typical clear cell renal cell carcinoma.

Molecular Features

Genetic profile VHL mutations have been identified in 25% of tumors.

Differential Diagnosis

The differential diagnosis mainly includes clear cell renal cell carcinoma with cystic degeneration, tubulocystic carcinoma of the kidney, cystic nephroma, clear cell-papillary renal cell carcinoma with predominant cystic configuration, and benign multilocular renal cortical cysts.
Fig. 1

The typical gross appearance of multilocular cystic renal neoplasm of low malignant potential characterized by variably sized cysts separated by thin septa and filled with clear, serous, and gelatinous fluid without nodules

References and Further Reading

  1. Suzigan, S., López-Beltrán, A., Montironi, R., et al. (2006). Multilocular cystic renal cell carcinoma : A report of 45 cases of a kidney tumor of low malignant potential. American Journal of Clinical Pathology, 125(2), 217–222.CrossRefGoogle Scholar
  2. Tretiakova, M., Mehta, V., Kocherginsky, M., Minor, A., Shen, S.S., Sirintrapun, S.J., Yao, J.L., Alvarado-Cabrero, I., Antic, T., Eggener, S.E., Picken, M.M., & Paner, G.P. (2018) Predominantly cystic clear cell renal cell carcinoma and multilocular cystic renal neoplasm of low malignant potential form a low-grade spectrum. Virchows Archiv, 473, 85–93. [Epub ahead of print].Google Scholar
  3. Williamson, S. R., Halat, S., Eble, J. N., Grignon, D. J., Lopez-Beltran, A., Montironi, R., Tan, P. H., Wang, M., Zhang, S., Maclennan, G. T., Baldridge, L. A., & Cheng, L. (2012). Multilocular cystic renal cell carcinoma: Similarities and differences in immunoprofile compared with clear cell renal cell carcinoma. The American Journal of Surgical Pathology, 36(10), 1425–1433.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