A Kidney Tumor in an Adolescent with Severe Hypertension and Hypokalemia: An Uncommon Case – Case Report and Review of the Literature on Reninoma

Gottardo, Fedra; Cesari, Maurizio; Morra, Aldo; Gardiman, Marina Paola; Fassina, Ambrogio; Bianco, M. Dal

doi:10.1159/000314339

Cited by 29 publications

(27 citation statements)

References 17 publications

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“…According to previous studies, the majority of reninomas present in the form of solitary tumor (11,18). The coexistence of reninoma and bilateral pheochromocytoma has been previously reported (19), whereas the present study is the first case report of reninoma coexisting with an adrenal adenoma.…”

Section: Discussionsupporting

confidence: 55%

Reninoma coexisting with adrenal adenoma during pregnancy: A case report

et al. 2017

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Abstract. Reninoma is a rare form of secondary hypertension. The present study reported a case of reninoma that coexisted with an adrenal adenoma in a young female with secondary hypertension during pregnancy. The patient, a 31-year-old female exhibiting hypertension for >1 year, developed a mass in the right adrenal gland, which was detected by an ultrasound scan in the 33rd gestational week. In addition, a well-defined solid mass on the left kidney was detected by a magnetic resonance imaging scan1 month subsequent to the termination of the pregnancy. The blood pressure (BP) of the patient did not change subsequent to the laparoscopic right adrenalectomy. The patient subsequently underwent laparoscopic left partial nephrectomy 2 months later. The BP levels demonstrated a significant reduction from 177/115 to 125/80 mmHg in the same day postoperatively, and stabilized to be within the normal range. The BP level of the patient remained within the normal range subsequent to a 6-month follow-up. Pathological analysis revealed reninoma on the left kidney. The present study aimed to provide information for radiologists who may encounter this type of benign tumor in the future, which exhibits elevated BP levels. IntroductionReninomas, or renal juxtaglomerular cell tumors, develop within the juxtaglomerular apparatus through the evolution of small artery smooth muscle cells. These tumor cells produce excessive amounts of renin that results in severe hypertension with hypokalemia and hyperaldosteronism, through the activity of the renin-angiotensin aldosterone system (1). It is a rare form of secondary hypertension. Removing the reninoma results in the conversion of increased blood pressure to normal blood pressure (2). Therefore, perioperative hemodynamic management is important in patients who undergo surgery for the removal of a reninoma.Adrenal adenoma is a benign neoplasm, which is derived from cells of the adrenal cortex, and may be functionally active or nonfunctional (3). Functional adrenal adenoma is able to cause aldosteronism or Cushing's syndrome. Primary aldosteronism (PA) is characterized by autonomous aldosterone production, which is a cause of secondary hypertension (4). Evidence has developed over the past decades and led to the recognition that autonomous adrenal aldosterone production, termed primary aldosteronism, is common in hypertensive patients. Between 5 and 13% of patients with hypertension have primary aldosteronism (5).The present study reported a rare case of reninoma coexisting with an adrenal adenoma in a young female with secondary hypertension during pregnancy. Case reportA 2.5-cm mass was detected on the right adrenal gland of a 31-year-old pregnant female with hypertension in her 33rd gestational week by an ultrasound scan. The patient presented with a personal history of elevated blood pressure (BP) for several months prior to conception, and exhibited a family history of hypertension. There were no other abnormalities found by the ultrasounds of the adrenal gland and the renal arter...

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Section: Discussionsupporting

confidence: 55%

Reninoma coexisting with adrenal adenoma during pregnancy: A case report

et al. 2017

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“…Since the first description in 1967 of a patient with severe hypertension resolving following removal of a cortical renal tumour found to contain large amounts of renin [1], reninomas appear to have been rarely reported, with only about 90 cases readily found in the literature [6,7]. They are more common in younger patients with most reports in those <25 years of age, and there is a female preponderance.…”

Section: Discussionmentioning

confidence: 99%

Reninoma: The Importance of Renal Vein Renin Ratios for Lateralisation and Diagnosis

Wolley¹,

Gordon²,

Stowasser³

2013

Am J Nephrol

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Background/Aim: Reninomas are rare juxtaglomerular tumours which can cause severe hypertension and hypokalaemia. Diagnosis can be problematic and these tumours can be difficult to locate on imaging. In this report we aim to demonstrate the value of carefully performed renal vein renin ratios (RVRRs) to assist in locating these tumours. Method/Results: We report on 3 patients diagnosed with reninoma in our unit. The patients were all female, young (17, 16 and 30 years), severely hypertensive and hypokalaemic (2.5, 2.5 and 3.1 mmol/l). Plasma renin activity (PRA) was elevated (31.9, 274 and 175 ng/ml/h), and aldosterone was high-normal (19.9 ng/dl) or elevated (207 and 109.3 ng/dl). Renal artery stenosis was excluded by renal artery Doppler, DTPA scan and angiography. Renal CT detected the lesion in 2 patients, with one lesion visible on pre- and post-contrast CT and the other on post-contrast CT only. RVRRs were performed several weeks after withdrawing interfering medications, maintaining a <40 mmol/day low-sodium diet and maintaining recumbency overnight the night before and during the procedure. Ratios before and after captopril or enalaprilat administration were obtained and lateralised the tumours in all 3 cases (dominant/non-dominant ratios of 2.3, 4.3 and 3.8). All of the patients underwent nephrectomy yielding a typical juxtaglomerular tumour and resulting in cure of hypertension and hypokalaemia. Conclusions: Reninoma should be suspected in young hypertensives (especially females) with significant hypokalaemia and high PRA or direct renin concentration after renovascular hypertension has been excluded. CT imaging and carefully performed RVRRs provide the highest likelihood of locating these tumours.

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“…The increasing availability of affordable genetic/molecular testing may elucidate further the pathogenesis of these rather rare genetic conditions and their implication in hypertension seen in the general public. Excessive production of aldosterone (primary: familial hypoaldosteronism, aldosterone-producing adenoma/ carcinoma/hyperplasia [13][14][15][16] or secondary: reninoma [17][18][19][20]) and sometimes of [ 7 ] . Z Glom zona glomerulosa; Z Fas zona fasciculata; Z Ret zona reticularis; 19-H 19-Hydroxylase; HSD Hydroxysteroid dehydrogenase; P450aro aromatase; 5alpha-Red 5alpha-Reductase.…”

Section: Pathogenesismentioning

confidence: 99%

Syndromes of Mineralocorticoid Excess

Melcescu

Koch

2012

Endocrine Hypertension

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In addition to obesity, diabetes type 2, and the metabolic syndrome, hypertension represents a major public and global health problem, most of which can be improved by lifestyle changes, including changing dietary habits with less consumption of processed and preserved foods, which generally contain higher amounts of salt than freshly prepared food items. Amongst causes for endocrine hypertension are syndromes of mineralocorticoid excess typically resulting from overactive amiloride-sensitive sodium channels located in the distal convoluted tubules and collecting ducts of the kidney, as well as other tissues, including vascular smooth muscle. The net effect of such an overactivation, which occurs mostly in primary aldosteronism is sodium and water retention with volume expansion and hypertension that is exacerbated by a diet high in salt. Biochemically, plasma renin activity is suppressed and hypokalemia may be present. Aldosterone mediates its action through the mineralocortoid receptor (MR), which regulates salt homeostasis in the kidneys and plays a range of other roles in the vasculature, heart, brain, and adipose tissue.Excessive MR activation can promote in fl ammation, fi brosis, and heart disease as well as psychiatric illness, including anxiety and depression, through key modulators, including the glucocorticoid receptor and the 11 b -hydroxysteroid dehydrogenases.Apart from aldosterone, the MR is also activated by products of abnormal adrenal steroid biosynthesis, dysregulated metabolism of cortisol in cells that are targets of mineralocorticoids, and activating mutations of the MR or of ion channels that are inducible by the MR. We provide here an overview of mineralocorticoid excess caused by congenital adrenal hyperplasia due to mutations of the 11beta-hydroxylase and 17alpha-hydroxylase genes, by mutations of the 11beta-hydroxysteroid dehydrogenase type 2 gene (apparent mineralocorticoid excess (AME)), mutations of the epithelial sodium channel genes (Liddle syndrome), mutations of the

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A Kidney Tumor in an Adolescent with Severe Hypertension and Hypokalemia: An Uncommon Case – Case Report and Review of the Literature on Reninoma

Cited by 29 publications

References 17 publications

Reninoma coexisting with adrenal adenoma during pregnancy: A case report

Reninoma coexisting with adrenal adenoma during pregnancy: A case report

Reninoma: The Importance of Renal Vein Renin Ratios for Lateralisation and Diagnosis

Syndromes of Mineralocorticoid Excess

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