Poster Abstract Abstract

(Paediatric), #220531305586

A case of a renin-secreting tumour in an adolescent: a rare yet curable cause of hypertension.

Meenal Mavinkurve, School of Medicine, International Medical University, Jalan Rasah, Seremban Negeri Sembilan; Department of Paediatrics, University Malaya Medical Centre, Kuala Lumpur 59100; Cheng Hooi Peng, Department of Paediatrics, University Malaya Medical Centre, Kuala Lumpur 59100; Department of Paediatrics, Hospital Putrajaya, Jln P9 Precinct 7, Putrajaya 62250; Man Kein Seong Mun Kein Seong, Department of Pathology, University Malaya Medical Centre, Kuala Lumpur 59100; Faculty of Medicine, University Malaya, Kuala Lumpur 50603; Norshazriman Sulaiman, Department of Biomedical Imaging, University Malaya Medical Centre, Kuala Lumpur 59100; Faculty of Medicine, University Malaya, Kuala Lumpur 50603; Nurshadia Samingan, Department of Paediatrics, University Malaya Medical Centre, Kuala Lumpur 59100; Faculty of Medicine, University Malaya, Kuala Lumpur 50603; Muhammad Yazid Jalaludin, Department of Paediatrics, University Malaya Medical Centre, Kuala Lumpur 59100; Faculty of Medicine, University Malaya, Kuala Lumpur 50603; Azriyanti Anuar Zaini, Department of Paediatrics, University Malaya Medical Centre, Kuala Lumpur 59100; Faculty of Medicine, University Malaya, Kuala Lumpur 50603

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Introduction

Reninomas are benign tumours of the juxtaglomerular apparatus that autonomously secrete renin. Only 100 cases have been reported in the literature. Adolescents with reninomas typically present with refractory hypertension that requires treatment multiple anti-hypertensives. However, hypertension secondary to reninomas are curable with surgery. Another, minimally invasive procedure, known as cryoablation has been successfully used to cure hypertension in an adult with reninoma, but this has not been reported in an adolescent.

Methods

We conducted a retrospective chart review of the pertinent clinical, biochemical, radiological and histopathological details.

Results

We report on a 14-year-old boy with a hypertensive emergency; blood pressure 180/100 mmHg and Bell’s palsy. His initial investigations showed hypokalaemia 2.2 mmol/L, metabolic alkalosis, raised plasma renin activity 2235mU/L and aldosterone 8056 pmol/L, suggesting a high-renin mineralocorticoid excess syndrome. A right-sided renal cortical cyst was seen on abdominal computed tomography, measuring 0.9 x 1.6 cm. In order to accurately establish lateralisation of the autonomous renin secretion, renal vein sampling (RVS) was conducted to determine renin ratios, which confirmed lateralisation to the right renal vein (ratio 2.72). His hypertension was difficult to control despite amlodipine, prazocin and verapamil and captopril. There were no complications. Following adequate optimisation of his hypertension, he underwent cryoablation of the lesion. The histopathology was conclusive for a juxtaglomerular tumour. One week post ablation, he had resolution of his hypertension and normalisation of the plasma renin activity to 13.4 mU/L after 1 month.

Conclusion

Reninoma, though rare, should be considered in adolescents who present with a triad of refractory hypertension, hypokalaemia and metabolic alkalosis. It is a curable with surgery, but cryoablation should be given due consideration. This case report illustrates that cryoablation can be used successfully for the management of reninoma in adolescents.

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