严重后果的良性肾肿瘤:1例青少年肾小球旁细胞瘤合并慢性肾脏疾病

IF 0.7 Q4 PEDIATRICS
Case Reports in Pediatrics Pub Date : 2025-06-13 eCollection Date: 2025-01-01 DOI:10.1155/crpe/9318115
Rachael Courtney, Erin Dahlinghaus, Abiodun Omoloja, Jeffrey T Boyd, Michelle Smith, Laura Biederman, Israel Ndengabaganizi, Daniel Keith Robie, Ahmad Al Dughiem
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引用次数: 0

摘要

肾小球旁细胞瘤(JGCT)是一种罕见的良性肿瘤。它的特点是严重的高血压和低钾血症,这是由于肾小球传入小动脉的肾小球旁器官产生过多的肾素所致。在此,我们报告一例15岁的女性患严重高血压的JGCT。发现肾素和醛固酮水平升高伴低钾血症,并对血管紧张素转换酶抑制剂(ACEi)有反应。腹部MRI示左侧肾肿瘤,直径4厘米。行根治性肾切除术,组织学显示肿瘤由多边形到卵形细胞片组成,与JGCT一致。术后肾素和醛固酮水平恢复正常,小剂量ACEi药物控制血压。不幸的是,在诊断之前,由于长期不受控制的高血压损害,患者一直处于III期慢性肾衰竭。我们在此回顾文献并讨论鉴别诊断。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Benign Renal Tumor With Serious Consequences: A Case Report of Juxtaglomerular Cell Tumor and Chronic Renal Disease in a Teenager.

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.

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自引率
11.10%
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审稿时长
13 weeks
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