Quach Priscilla, Hamza Ameer
University of Kansas Medical Center, Department of Pathology & Laboratory Medicine, Kansas City, Kansas, USA.
Autops Case Rep. 2022 Oct 21;12:e2021406. doi: 10.4322/acr.2021.406. eCollection 2022.
Juxtaglomerular cell tumor is a benign, renin-secreting neoplasm. The tumor arises from the juxtaglomerular apparatus cells of the kidney. Because the tumor is hormonally active, patients usually suffer from hypokalemia, hyperaldosteronism, and hypertension. Herein, we describe a case of a 19-year-old Asian female with a somewhat unusual presentation. A 19-year-old Asian female presented with upper extremity weakness, numbness, and tingling. On physical examination, the only notable finding was hypertension. Extensive workup revealed elevated aldosterone level and plasma renin activity. CT scan of the abdomen revealed a 2.2 cm mass in the lower pole of the left kidney. The mass was resected by partial nephrectomy. On microscopic evaluation, the tumor had glomoid appearance with sheets of uniform, round to polygonal cells with clear to eosinophilic cytoplasm. Immunohistochemical stains showed the tumor cells to be positive for CD117, CD34 and CD10 and negative for ER, PR, CK7, PAX-8, pan-cytokeratin, EMA, S100, Melan-A, HMB45, SMA and CAIX. Diagnosis of Juxtaglomerular cell tumor was rendered. This case highlights the importance of a regular physical exam and a high index of suspicion in patients presenting with unusual complaints.
球旁细胞瘤是一种良性的、分泌肾素的肿瘤。该肿瘤起源于肾脏的球旁器细胞。由于肿瘤具有激素活性，患者通常会出现低钾血症、醛固酮增多症和高血压。在此，我们描述一例19岁亚洲女性患者，其临床表现有些不寻常。一名19岁亚洲女性出现上肢无力、麻木和刺痛。体格检查时，唯一显著的发现是高血压。全面检查发现醛固酮水平升高和血浆肾素活性升高。腹部CT扫描显示左肾下极有一个2.2 cm的肿块。通过部分肾切除术切除了肿块。显微镜评估显示，肿瘤呈肾小球样外观，有一片片均匀的圆形至多边形细胞，细胞质清晰至嗜酸性。免疫组化染色显示肿瘤细胞CD117、CD34和CD10呈阳性，而雌激素受体（ER）、孕激素受体（PR）、细胞角蛋白7（CK7）、配对盒基因8（PAX-8）、全细胞角蛋白、上皮膜抗原（EMA）、S100、黑素A、HMB45、平滑肌肌动蛋白（SMA）和碳酸酐酶IX（CAIX）呈阴性。诊断为球旁细胞瘤。该病例强调了定期体检以及对有异常症状患者保持高度怀疑指数的重要性。
