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.
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.
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.
Juxtaglomerular cell tumor is often well-circumscribed, yellow to gray-tan in color, with a complete or partial fibrous capsule usually observed. Histologically, it consists of solid sheets of closely packed round to polygonal cells.
Imaging findings are variable.
- hypoechoic mass
- variable density with moderate enhancement during late phase after contrast administration
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.
On imaging consider other renal tumors such as
- glomus tumor - kidney
- hemangiopericytoma - kidney
- metanephric adenoma - kidney
- papillary renal cell carcinoma
- collecting duct carcinoma
- urothelial carcinoma
- renal angiomyolipoma
- Wilms tumor
- 1. Robertson PW, Klidijan A, Harding LK, Walters G, Lee MR, Robb-Smith AHT: Hypertension due to a renin-secreting renal tumor. Am J Med 1967, 43(6):963-976.
- 2. Martin SA, Mynderse LA, Lager DJ et-al. Juxtaglomerular cell tumor: a clinicopathologic study of four cases and review of the literature. Am. J. Clin. Pathol. 2001;116 (6): 854-63. doi:10.1309/B10J-FKQ5-J7P8-WKU4 - Pubmed citation
- 3. Kim HJ, Kim CH, Choi YJ et-al. Juxtaglomerular cell tumor of kidney with CD34 and CD117 immunoreactivity: report of 5 cases. Arch. Pathol. Lab. Med. 2006;130 (5): 707-11. Pubmed citation
- 4. Rubenstein JN, Eggener SE, Pins MR et-al. Juxtaglomerular apparatus tumor: a rare, surgically correctable cause of hypertension. Rev Urol. 2011;4 (4): 192-5. Free text at pubmed - Pubmed citation