Juxtaglomerular cell tumours, also known as reninomas, are uncommon renal tumour of the juxtaglomerular cells. The tumour cells secrete renin and often cause severe hypertension and hypokalaemia.
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Epidemiology
Juxtaglomerular cell tumour 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 tumour present with headaches, dizziness, double vision, retinopathy, nausea, vomiting, and polyuria and most of these may be attributed to hypertension or hypokalaemia. Reninoma may be a reason of cerebrovascular accident and death 5.
Pathology
Juxtaglomerular cell tumour is often well-circumscribed, yellow to gray-tan in colour, with a complete or partial fibrous capsule usually observed. Histologically cytoplasm of tumour 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 tumour resection by radical or partial nephrectomy is the best treatment for juxtaglomerular cell tumour. Anti-hypertensive agents can be used to manage hypertension until definitive therapy is planned.
History and etymology
Juxtaglomerular cell tumour 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 tumours such as
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