Courtney Rachael, Dahlinghaus Erin, Omoloja Abiodun, Boyd Jeffrey T, Smith Michelle, Biederman Laura, Ndengabaganizi Israel, Robie Daniel Keith, Al Dughiem Ahmad
Departments of Pediatrics, Dayton Children's Hospital and Wright State University Boonshoft School of Medicine, Dayton, Ohio, USA.
Department of Pathology, Dayton Children's Hospital and Wright State University Boonshoft School of Medicine, Dayton, Ohio, USA.
Case Rep Pediatr. 2025 Jun 13;2025:9318115. doi: 10.1155/crpe/9318115. eCollection 2025.
Juxtaglomerular cell tumor (JGCT), or reninoma, is a rare benign renal neoplasm. It is characterized by severe hypertension and hypokalemia due to excessive renin produced from the juxtaglomerular apparatus on the afferent arteriole of the glomerulus. Herein, we report a case of JGCT in a 15-year-old female who presented with severe hypertension. She was found to have elevated levels of renin and aldosterone with hypokalemia and she responded to angiotensin-converting enzyme inhibitors (ACEi). Abdominal MRI demonstrated a 4-cm left renal tumor. A radical nephrectomy was performed, and histology showed a well-circumscribed tumor consisting of sheets of polygonal to ovoid cells consistent with a JGCT. After surgery, the renin and aldosterone levels normalized, and blood pressure was controlled with small dose of ACEi medication. Unfortunately, the patient remained in Stage III chronic kidney failure due to the longstanding damage of uncontrolled hypertension prior to the diagnosis. We hereby review the literature and discuss the differential diagnosis.
肾小球旁细胞瘤（JGCT），又称肾素瘤，是一种罕见的良性肾肿瘤。其特征为严重高血压和低钾血症，这是由于肾小球入球小动脉上的肾小球旁器分泌过多肾素所致。在此，我们报告一例15岁患有严重高血压的女性JGCT病例。她被发现肾素和醛固酮水平升高，伴有低钾血症，且对血管紧张素转换酶抑制剂（ACEi）有反应。腹部MRI显示左肾有一个4厘米的肿瘤。进行了根治性肾切除术，组织学检查显示肿瘤边界清晰，由成片的多边形至卵圆形细胞组成，符合JGCT。手术后肾素和醛固酮水平恢复正常，小剂量ACEi药物控制了血压。不幸的是，由于诊断前长期未控制的高血压造成的长期损害，该患者仍处于III期慢性肾衰竭。我们在此回顾文献并讨论鉴别诊断。
