Juxtaglomerular Cell Tumor of the Kidney
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.
Juxtaglomerular cell tumor is very rare with approximately 100 cases reported in the literature.
The tumor typically occurs in young adults with a peak incidence in the second to fourth decades.
There is a slight female predominance.
Partial or radical nephrectomy is the standard of care.
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.
Juxtaglomerular cell tumor is a yellow-tan mass circumscribed by a fibrous capsule.
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.
Neoplastic cells are positive for renin, vimentin, actin, CD34, and CD117 (Kim et al. 2006).
Cytogenetic analyses show loss of chromosome 9 and 11 as well as aneusomy (Capovilla et al. 2008).
References and Further Reading
- 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