Juxtaglomerular Cell Tumor of the Kidney

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...