Juxtaglomerular cell tumors, also known as reninomas, are uncommon renal tumor of the juxtaglomerular cells. The tumor cells secrete renin and often cause severe hypertension and hypokalemia.
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Epidemiology
Juxtaglomerular cell tumor affect all age groups, but are most common in adolescents and young adults, with peak prevalence in the second and third decades of life.
There is a female predominance.
Clinical presentation
Patients with a juxtaglomerular cell tumor present with headaches, dizziness, double vision, retinopathy, nausea, vomiting, and polyuria and most of these may be attributed to hypertension or hypokalemia. Reninoma may be a reason of cerebrovascular accident and death 5.
Pathology
Juxtaglomerular cell tumor is often well-circumscribed, yellow to gray-tan in color, with a complete or partial fibrous capsule usually observed. Histologically cytoplasm of tumor cells consisting of renin and solid sheets of closely packed round to polygonal cells 5.
Radiographic features
Imaging findings are variable.
Ultrasound
hypoechoic mass
CT
variable density with moderate enhancement during late phase after contrast administration
MRI
Reported signal characteristics include
T1: iso-signal intensity
T2: high-signal intensity
Treatment and prognosis
Complete tumor resection by radical or partial nephrectomy is the best treatment for juxtaglomerular cell tumor. Anti-hypertensive agents can be used to manage hypertension until definitive therapy is planned.
History and etymology
Juxtaglomerular cell tumor was originally described in 1967 by Robertson et al, but first named by Kihara et al. in 1968. Approximately 100 case reports have been published.
Differential diagnosis
On imaging consider other renal tumors such as
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