Encyclopedia of Pathology

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

Juxtaglomerular Cell Tumor of the Kidney

  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Living reference work entry
DOI: https://doi.org/10.1007/978-3-319-28845-1_4835-1

Definition

Juxtaglomerular cell tumor is a rare neoplasm secreting renin. It arises from the specialized smooth muscle of the glomerular afferent arteriole in the juxtaglomerular apparatus of the kidney.

Clinical Features

  • Incidence

    Juxtaglomerular cell tumor is very rare with approximately 100 cases reported in the literature.

  • Age

    The tumor typically occurs in young adults with a peak incidence in the second to fourth decades.

  • Sex

    There is a slight female predominance.

  • Treatment

    Partial or radical nephrectomy is the standard of care.

  • Outcome

    Because the tumor secretes renin, the patients present with severe and poorly controlled hypertension, hyperaldosteronism, and hypokalemia. Usually, the clinical symptoms resolve after surgical removal. These tumors usually have an indolent course.

Macroscopy

Juxtaglomerular cell tumor is a yellow-tan mass circumscribed by a fibrous capsule.

Microscopy

The tumor is composed of uniform polygonal to spindle cells with central round uniform nuclei and variable amounts of eosinophilic cytoplasm, arranged in papillary architecture. Often, entrapped renal tubules are present. The tumor contains a prominent vascular network with thin-wall vessels and thick-wall vessels, frequently hyalinized (Kuroda et al. 2013). Hemangiopericytomatous vascular pattern can be seen. Cytological atypia, necrosis, and mitoses are uncommon.

Immunophenotype

Neoplastic cells are positive for renin, vimentin, actin, CD34, and CD117 (Kim et al. 2006).

Molecular Features

Cytogenetic analyses show loss of chromosome 9 and 11 as well as aneusomy (Capovilla et al. 2008).

References and Further Reading

  1. Capovilla, M., Couturier, J., Molinié, V., et al. (2008). Loss of chromosomes 9 and 11 may be recurrent chromosome imbalances in juxtaglomerular cell tumors. Human Pathology, 39, 459–462.CrossRefGoogle Scholar
  2. Kim, H. J., Kim, C. H., Choi, Y. J., et al. (2006). Juxtaglomerular cell tumor of kidney with CD34 and CD117 immunoreactivity: Report of 5 cases. Archives of Pathology & Laboratory Medicine, 130, 707–711.Google Scholar
  3. Kuroda, N., Maris, S., Monzon, F. A., et al. (2013). Juxtaglomerular cell tumor: A morphological, immunohistochemical and genetic study of six cases. Human Pathology, 44, 47–54.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