Reninoma: an uncommon cause of Renin-mediated hypertension.

Peter Trnka,Luisa Orellana,Peter Borzi,Mark Walsh,Louis Pool

doi:10.3389/fped.2014.00089

Abstract

Reninoma (juxtaglomerular cell tumor) is a rare cause of renin-mediated hypertension. Reninomas are usually diagnosed in adolescents and young adults with occasional reports in younger children. Patients typically present with a long history of headaches leading to a diagnosis of severe hypertension that responds well to antihypertensive treatment targeting the renin-angiotensin-aldosterone system. The clue to clinical diagnosis is the presence of hypokalemia and metabolic alkalosis on the first blood sample drawn before any treatment is instituted. Elevated blood levels of renin and aldosterone confirm the clinical suspicion of renin-mediated hypertension. Diagnostic imaging is employed to identify the source of excessive renin production. While renal ultrasound can miss reninoma, contrast CT or magnetic resonance imaging of the kidneys are diagnostic modalities of choice leading to the correct diagnosis. Renal vein renin sampling with lateralization might help to identify the site of excessive renin production. Nephron-sparing surgery is curative with maintenance of normal blood pressure after discontinuation of antihypertensive medications in the majority of patients. In this paper, we present the case of reninoma in an adolescent girl emphasizing clinical presentation, diagnostic evaluation, and medical and surgical treatment of this rare tumor. We also discuss important points in the management of children presenting with renin-mediated hypertension.

Highlights

Prevalence of hypertension in children is about 2.5–3%, with some studies suggesting an increasing trend, making elevated blood pressure one of the most common conditions in childhood [1]
We present the case of reninoma in an adolescent girl emphasizing clinical presentation, diagnostic evaluation, and medical and surgical treatment of this rare tumor
We describe the case of reninoma in an adolescent girl, who presented with a long history of headaches suggestive of renin-mediated hypertension

Summary

Introduction

Prevalence of hypertension in children is about 2.5–3%, with some studies suggesting an increasing trend, making elevated blood pressure one of the most common conditions in childhood [1]. Severe hypertension (usually defined as blood pressure at least 20 mmHg above the 95% percentile for age, sex, and height) requires immediate medical attention with gradual reduction of blood pressure and thorough investigations for the secondary causes of hypertension [2]. Kidney disease is the most common cause of secondary hypertension – one of the reasons why pediatric nephrologists are usually involved in the management of these children. Increased production of renin from the affected kidneys causes blood pressure elevation, leading to the development of renin-mediated hypertension. Reninomas are an uncommon but welldescribed cause of renin-mediated hypertension with about 100 reported cases to date [3]

Discussion

Conclusion