Juxtaglomerular Cell Tumor: Reviewing a Cryptic Cause of Surgically Correctable Hypertension

Abstract

Juxtaglomerular cell tumor (JGCT), or reninoma, is a typically benign neoplasm generally affecting adolescents and young adults due to modified smooth muscle cells from the afferent arteriole of the juxtaglomerular apparatus. Patients experience symptoms related to hypertension and hypoka-lemia due to renin-secretion by the tumor. MRI, PET, CT, and renal vein catheterizations can be used to capture JGCTs, with laparoscopic ultrasonography being most cost-efec-tive. Surgical removal is the best option for management; electrolyte imbalances are a potential complication which may be assuaged via pre-surgical administration of aliskiren, a renin inhibitor. Considering the vast etiology for hypertension and rarity of JGCT, the diagnosing physician must have a high index of suspicion for JGCT. Early recognition and management can help prevent cardiovascular or pregnancy complications and fatalities, vascular invasion and metastasis, improve quality of life, and limit socioeconomic liabilities. Herein we review the epidemiology, genetics, histopathol-ogy, clinical presentation, and management of this rare condition. The impact of genetics on prognosis warrant further research.

Juxtaglomerular cell tumour of the kidney: a rare cause of resistant hypertension

“…However, since today, five cases with malignant characteristics have been described. Their malignant potency was confirmed either by metastases in solid organs (liver and lungs) or by tumour recurrence and vascular invasion on histopathology (5). Furthermore, in one of these cases, the patient presented with paraneoplastic syndrome.…”

“…Laparoscopic approach is mainly recommended as the tumours are usually small and superficial and consequently easily accessible. However, in cases of large or deep, centrally located tumours in which nephron-sparing surgery is not feasible, radical nephrectomy is recommended for a complete cure (5). Besides the surgical approach, in some patients who were unwilling or unsuitable for surgery or before the surgical approach, pharmacologic management of JGCT with a renin inhibitor, aliskiren, has been proved to be beneficial.…”

Vascular Neoplasms and Related Lesions of Kidney

“…It is now widely recognized as a rare cause of hypertension and secondary hyperaldosteronism, with approximately 170 cases reported in the literature to date [100]. JGCT typically occurs in young adults; it is twice as common in women and has a peak incidence in the second to third decade of life [15,101]. Most cases present with hypertension and its related symptoms (such as headache, dizziness, nausea and vomiting) along with hypokalemia, but atypical JGCTs (presenting with hypertension and normokalemia) and very rarely, nonfunctioning JGCTs (presenting with normotension and normokalemia) have also been reported [15,102,103].…”

“…The mainstay of management of JGCT is complete tumor resection, which usually leads to normalization of blood pressure and renin levels [103]. Most JGCTs display benign behavior, but nevertheless patients can experience significant morbidity and complications related to hypertension including retinopathy, congestive heart failure, renal failure, intestinal ischemia, and brain hemorrhage [101,103,105,106]. Three cases of metastatic JGCT have been reported; two cases documented bilateral lung metastasis and one case documented synchronous liver and spleen metastasis at presentation [102,106,108].…”

Juxtaglomerular cell tumor diagnosed preoperatively by renal tumor biopsy

“…JGCT is one kind of the diseases which can present small renal mass 4 . Since most of the JGCT patients show characteristic symptoms, such as hypertension and hypokalemia, due to oversecretion of renin from the tumor, diagnosis of the disease had been made mainly based on the presence of the characteristic symptom and radiographic findings.…”