Ohashi Yasushi, Kobayashi Shizuka, Arai Taichi, Nemoto Tetsuo, Aoki Chizu, Nagata Masato, Sakai Ken
Department of Nephrology, Faculty of Medicine, Toho University, Tokyo, Japan.
Department of Surgical Pathology, Faculty of Medicine, Toho University, Tokyo, Japan.
Case Rep Nephrol Urol. 2014 May 7;4(2):88-94. doi: 10.1159/000362757. eCollection 2014 May.
Juxtaglomerular cell tumor is a rare renal neoplasm. Secondary hypertension with juxtaglomerular cell tumor can be seen in females in their 20s and 30s. We present a case of juxtaglomerular cell tumor during pregnancy. A 32-year-old female was hospitalized for refractory hypertension and nephrotic syndrome in the 23rd gestational week. One year before admission, she had been diagnosed with hypertension; plasma renin activity at that time had been 2.3 ng/ml/h. Her blood pressure was uncontrolled during pregnancy, and proteinuria was detected in the 12th gestational week despite the administration of antihypertensive medications. Laboratory data showed proteinuria, hypokalemia, and hypoalbuminemia. In the 25th gestational week, she underwent surgical termination of the pregnancy because of congestive heart failure and acute renal injury. After the termination of the pregnancy and the delivery of a viable fetus, her hypertension and nephrotic syndrome were found to persist with a high plasma renin activity (13 ng/ml/h). Ultrasonography showed a 5.5-cm left renal cystic mass with a partially solid component at the lower renal pole. The left kidney with the renal mass was excised by laparoscopic nephrectomy. Plasma renin activity normalized the next day, with a decrease in blood pressure to 120-130/80-90 mm Hg; however, proteinuria remained at ≥3.5 g/day. On the basis of histopathological findings, the patient was diagnosed with a juxtaglomerular cell tumor and focal segmental glomerulosclerosis. Juxtaglomerular cell tumor is a rare renin-secreting tumor associated with refractory hypertension in young females and is a possible cause of hypertension during pregnancy.
球旁细胞瘤是一种罕见的肾脏肿瘤。20多岁和30多岁的女性可见球旁细胞瘤伴发继发性高血压。我们报告一例妊娠期球旁细胞瘤病例。一名32岁女性在妊娠第23周因难治性高血压和肾病综合征住院。入院前一年,她被诊断为高血压;当时血浆肾素活性为2.3 ng/ml/h。她在孕期血压控制不佳,尽管使用了降压药物,但在妊娠第12周仍检测到蛋白尿。实验室检查显示有蛋白尿、低钾血症和低白蛋白血症。在妊娠第25周,由于充血性心力衰竭和急性肾损伤,她接受了妊娠终止手术。终止妊娠并娩出一个存活胎儿后,发现她的高血压和肾病综合征持续存在,血浆肾素活性较高(13 ng/ml/h)。超声检查显示左肾下极有一个5.5 cm的肾囊性肿块,部分为实性成分。通过腹腔镜肾切除术切除了带有肾肿块的左肾。第二天血浆肾素活性恢复正常,血压降至120 - 130/80 - 90 mmHg;然而,蛋白尿仍≥3.5 g/天。根据组织病理学检查结果,患者被诊断为球旁细胞瘤和局灶节段性肾小球硬化。球旁细胞瘤是一种罕见的分泌肾素的肿瘤,与年轻女性的难治性高血压有关,是妊娠期高血压的一个可能原因。