腎素瘤合併術後持續高血壓與腎功能異常- 一個罕見的病例報告

李采霓、黃國皓

國立台灣大學附設醫院 泌尿部

Persistent hypertension and impaired renal function despite successful resection of a reninoma – a rare case report

Tsae-Ni, LeeKuo-How Huang

Department of Urology, National Taiwan University Hospital 

 

Abstract

Introduction:

A reninoma, also known as a juxtaglomerular cell tumor (JGCT), is a rare renal tumor that secretes renin, further causing secondary hypertension. Surgical resection of the tumor is the optimal treatment, and previous literature has shown improvement of the patient’s blood pressure after tumor resection. The first reported JGCT resected through robotic surgery was in 2020 (1), by urologists in Chung-Ang University College of Medicine, Seoul, Korea. Here, we present a case of reninoma that was resected by a robot-assisted partial nephrectomy.

 

Case presentation:

A 41-year-old Taiwanese woman with recurrent episodes of hypertensive crisis and acute decompensated heart failure for 3 years, presented to us for further evaluation and treatment. Previous laboratory examinations at an outside hospital revealed an elevated plasma renin activity, renin level, Chromogranin A level, proteinuria, leukocytosis, anemia, elevated pro BNP, impaired renal function, hyponatremia, and hypokalemia. The serum aldosterone level was within normal limits. A cardiac echo showed a grossly normal left ventricle with a preserved ejection fraction (=53.20%). An abdominal MRI revealed a 1.7 cm T1 isointense, T2 hypointense tumor at the upper pole of the right kidney (Figure 1). A computed tomography-guided renal tumor biopsy was then performed and the pathology reported a juxtaglomerular cell tumor, also known as reninoma. Considering her multiple comorbidities, recurrent episodes of acute decompensated heart failure along with renal function impairment, surgery was arranged as soon as her condition was favorable. She received a robot-assisted right partial nephrectomy smoothly without any complications. The resected tumor was 1.6 x 1.2 x 1.1 cm in size, flesh colored, well-defined, and grossly confined to the kidney (figure 2). The pathology reported a juxtaglomerular cell tumor. Her serum potassium and sodium level returned to within normal range after the surgery, however, oliguria, impaired renal function and hypertension were still noted, despite the number of anti-hypertensive medications used were less when compared to pre-surgical status. A weekly hemodialysis was still needed, even 7 months after the surgery, for severe impaired renal function was still noted.
 
Discussion:

The initial presentation of reninoma in our case was hypertension, which was difficult to control. Her condition gradually worsened, and heart failure developed. In addition, renal failure, which is implied to be due to cardiorenal syndrome, also occurred. And despite our case’s reninoma was successfully removed via surgery, and that the postoperative lab data showed normalization of her serum potassium and sodium levels, impaired renal function was still noted and she still needed hemodialysis. In addition, the blood pressure of the cases of reninoma patients reported in previous studies all normalized after a surgical resection of the reninoma, however, in our case, despite the dosage and types of antihypertensive medications did decrease after the reninoma was resected, our patient still needed an angiotensin II receptor blocker, a beta-blocker, and a calcium channel blocker to control her blood pressure.

As far as we know, this is the first case whose hypertension did not cure after the reninoma was resected. Furthermore, no case has been reported to suffer from prolonged renal damage, leading to hemodialysis.

 

Conclusion:

The cause of a secondary hypertension can be tricky to diagnose, for many various etiologies, such as a pheochromocytoma, or despite rare, a reninoma, may present with an elevated blood pressure. Urologists must always be alert of a different diagnosis, especially when encountering refractory hypertension, hypokalemia, and even acute decompensated heart failure. In our case, we successfully removed a reninoma via robot-assisted partial nephrectomy. Previous published cases showed a normalization of the patient’s plasma renin, serum potassium levels, and blood pressure without the usage of any antihypertensive medications after the renin-secreting tumor was resected. However, despite a decrease of the dosage and numbers of antihypertensive medications taken, the patient still needed 3 types of antihypertensive medications to control her blood pressure after the surgery. In addition, minimal improvement of her renal function was noted despite the tumor being resected. Could delayed treatment of a renin-secreting tumor possibly lead to permanent damage in the patient’s kidney and even cardiac function? Despite this, a robot-assisted partial nephrectomy was shown in our case to be a successful approach for a resection of a renin-secreting tumor.
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    台灣泌尿科醫學會
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    2023-01-03 21:39:39
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    2023-01-03 21:40:32
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