Characterization and Management of Juxtaglomerular Cell Tumor: Analysis of 9 Cases and Literature Review

Abstract

Aims: Juxtaglomerular cell tumor is a rare kidney tumor. This study aimed to report the clinic features of juxtaglomerular cell tumor and our treatment experience. Methods: The medical records of 9 juxtaglomerular cell tumor patients treated in our hospital from 1997 to 2017 were retrospectively reviewed. Clinical characteristics, immunohistochemical findings, treatments and outcomes were collected. Results: The mean age of 9 patients was 24±8.1 years (range: 18-37). All cases had symptoms of hypertension, hyperaldosteronism, high plasma renin, high plasma angiotensin II. Four cases had hypokalemia. The renal masses were found by enhanced contrast tomography in all patients. One case received ultrasound-guided ablation and was clinically diagnosed with juxtaglomerular cell tumor. Among the remaining 8 cases, 2 cases received nephrectomy while 6 underwent partial nephrectomy. The 8 cases were pathologically diagnosed with juxtaglomerular cell tumor. Immunohistochemical findings showed that juxtaglomerular cell tumor was positive for vimentin, CD34, and actin but negative for chromogranin A. After treatment, all the patients had normal levels of blood pressure, serum renin activity, potassium, and aldosterone. No patients had tumor progress or metastasis within a median follow-up period of 94 (range: 33-241) months. Conclusion: Hypertension combined with hyperaldosteronism and hypokalemia secondary to high plasma renin activity are the typical symptoms of juxtaglomerular cell tumor. Partial nephrectomy is an optimal treatment for juxtaglomerular cell tumor.

Juxtaglomerular cell tumor: Report of a case with unusual presentation

“…Although the tumor mainly affects young adults, cases have been reported in children and the elderly with an age range of 6-69 years. 1,[4][5][6] The prototypical presentation of a young female with unexplained hypertension was also seen in our case. However, the presenting symptoms of upper extremity weakness, numbness, and tingling were somewhat unusual.…”

“…Other reported symptoms include headache, vomiting, dizziness, nocturia, and polyuria. [2][3][4] Data from published cases and reviews suggest that the tumor frequently affects adolescents and young adults with a mean age 27 years at diagnosis and is twice as common in women as in men. Although the tumor mainly affects young adults, cases have been reported in children and the elderly with an age range of 6-69 years.…”

“…1 Because the tumor is hormonally active, patients usually suffer from hypokalemia, hyperaldosteronism, and hypertension. [1][2][3][4] The typical patient population is adolescents and young adults, with a slight predominance in females. [1][2][3][4] The most common clinical presentation is that of a young adult with severe hypertension refractory to medical therapy.…”

“…[1][2][3][4] The typical patient population is adolescents and young adults, with a slight predominance in females. [1][2][3][4] The most common clinical presentation is that of a young adult with severe hypertension refractory to medical therapy. Elevated levels of aldosterone and renin coupled with a renal mass on imaging are the mainstay of diagnosis, which is eventually confirmed histologically following resection of the mass.…”

“…Elevated levels of aldosterone and renin coupled with a renal mass on imaging are the mainstay of diagnosis, which is eventually confirmed histologically following resection of the mass. [2][3][4] Herein, we describe a case of a 19-year-old Asian female with a somewhat atypical presentation. This case highlights the importance of a regular physical exam and having a high index of suspicion in patients presenting with unusual complaints.…”