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A Syngeneic Mouse Model of Metastatic Renal Cell Carcinoma for Quantitative and Longitudinal Assessment of Preclinical Therapies
Published on: April 12, 2017
Approach to the Patient: Reninoma
Annabelle G Hayes1,2, Michael Stowasser3,4, Mahesh M Umapathysivam1,2
1Endocrine and Metabolic Unit, Royal Adelaide Hospital, Adelaide, South Australia 5000, Australia.
Abstract:
A reninoma is a functional tumor of afferent arteriolar juxtaglomerular cells that secretes the enzyme renin, leading to hyperactivation of the renin-angiotensin-aldosterone system. Reninoma is a potentially curable cause of pathological secondary hyperaldosteronism that results in often severe hypertension and hypokalemia. The lack of suppression of plasma renin contrasts sharply with the much more common primary aldosteronism, but diagnosis is often prompted by screening for that condition. The major differential diagnosis of reninoma is renovascular hypertension. Fewer than 200 cases of reninoma have been described. Reninomas have been reported across a broad demographic but have a 2:1 predilection for women, often of childbearing age. Aldosterone receptor blockade, angiotensin-converting enzyme inhibitors, or angiotensin receptor blockers offer effective medical management but are contraindicated in pregnancy, so surgical curative resection is ideal. The current optimal imaging and biochemical workup of reninoma and management approach (ideally, tumor excision with subtotal renal resection) are described.
Insights
Reninoma, a rare renin-secreting tumor, causes severe hypertension and hypokalemia. Surgical resection offers a cure for this secondary hyperaldosteronism, distinguishing it from primary aldosteronism.
Area of Science:
- Nephrology
- Endocrinology
- Oncology
Background:
- Reninoma is a rare, functional tumor of juxtaglomerular cells.
- It secretes renin, causing renin-angiotensin-aldosterone system (RAAS) hyperactivation.
- This leads to secondary hyperaldosteronism, severe hypertension, and hypokalemia.
Purpose of the Study:
- To describe the optimal diagnostic and management strategies for reninoma.
- To differentiate reninoma from primary aldosteronism and renovascular hypertension.
- To highlight surgical resection as the ideal curative treatment.
Main Methods:
- Review of clinical presentation, biochemical findings, and imaging characteristics of reninoma.
- Discussion of differential diagnoses, including primary aldosteronism and renovascular hypertension.
- Outline of current management approaches, emphasizing surgical tumor excision.
Main Results:
- Reninoma diagnosis is often prompted by screening for primary aldosteronism due to lack of plasma renin suppression.
- Renovascular hypertension is a key differential diagnosis.
- Fewer than 200 cases reported, with a 2:1 female predilection, often in childbearing age.
Conclusions:
- Reninoma is a curable cause of secondary hyperaldosteronism.
- Optimal management involves accurate diagnosis through imaging and biochemical workup.
- Surgical resection is the preferred curative treatment, especially given contraindications for medical management in pregnancy.
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