肾素瘤的诊断及外科处理

Diagnosis and surgical management of juxtaglomerular cell tumor of the kidney

目的 提高对肾素瘤的诊断与治疗水平。方法 回顾分析3例肾素瘤患者资料，并结合文献复习，探讨肾素瘤的临床特点、诊断程序及方法、外科处理原则。例1，女，15岁，因间断头痛、头晕1年、视物模糊3周入院。入院时血压245／135mmHg（1mmHg=0．133kPa），血钾2．5～3．0mmol／L。卧位及立位血浆肾素（PRA）2．2μg·L^-1·h^-1[正常值（0．42±0．37）μg·L^-1·h^-1]、3．5ug·L^-1·h^-1[正常值（2．97±1．02）μg·L^-1·h^-1]；血管紧张素II（ATII）181．2ng／Lm[E常值（40．2±12．0）ng／L]、481．4ng／L[-正常值（85．3±30．0）ng／L]；醛固酮（ALD）332、4pmol／L [正常值（238．2±103．8）pmol／L]、747．9pmol／Lm正常值（418．2±243．7）pmol／L]。B超及平扫CT未见明显病变。动态增强CT检查：门脉及延迟期右肾中部肾组织内见1．3cm类圆形稍低密度影，边缘较整齐。例2，男，39岁，因发现高血压11年、低血钾9年人院。血压180／120mmHg，血钾2．7～3．0mmol／L。卧位及立位PRA8．1、9．2μg·L^-1·h^-1，ATII198．3、279．1ng／L，ALD285．3、761．7pmol／L。B超及平扫CT未见明显病变。动态增强CT检查：门脉及延迟期右肾上部皮质内见1．2cm稍低密度影，略向肾外突出。例3，女，26岁，因高血压5个月、发现左肾占位1周人院。血压210／120mmHg，血钾4．1mmol／L。卧位及立位PRA0．1、0．3μg·L^-1·h^-1，ATII56．2、71．5ng／L，ALD321．3、421．1pmol／L。肾脏B超：左肾上极皮质2．8cm×1．6cm中强回声。动态增强CT：左肾上极3．0cm等密度肿物，增强扫描轻度强化。例1、2术前诊断右肾肾素瘤，例3术前诊为左肾肿瘤性质待查。均行肾部分切除术，其中例2为后腹腔镜下手术。结果 手术顺利，手术时间分别为90、50、70min，出血量120、40、220ml。病理：肿瘤有完整纤维包膜，肿瘤细胞成片排列，均匀一致，细胞多角形，核居中，胞质宽，嗜酸性，间质血管丰富，可见厚壁血管，符合肾素瘤。免疫组化：肌动蛋白（＋），CD34（＋）。术后血压均恢复正常，分别随访23、4、26个月，未见肿瘤复发与转移。结论 高血压、高肾素、继发性醛固酮增高、低血钾（三高一低）是肾素瘤特征性的临床表现。动态增强CT检查敏感性高，对诊断帮助大。肾部分切除术或肿瘤剜除术是有效的治疗手段。腹腔镜手术同开放手术一样安全有效。

Objective To improve the diagnosis and treatment quality of juxtaglomerular cell tumor of the kidney. Methods Three patients（2 females,1 male） were diagnosed with juxtaglomer ular cell tumor of kidney and underwent nephron-sparing surgery. Case 1 was female, 15 years old. She presented with hypertention of 245/135 mm Hg. The serum kalium was 2.5-3.0 mmol/L. The laboratory examination suggested that in decubitus and standing position, the plasma renin activity （PRA） was 2.2 and 3.5μg ·L^-1·h^-1 , angiotensin Ⅱ （ATⅡ） was 181.2 and 481.4 ng/L;aldosterone（ALD） was 332.4 and 747.9 pmol/L, respectively. Dynamic enhanced CT scanning demonstrated a tumor with the diameter of 1. 3 cm in right kidney. Case 2 was male, 39 years old. He presented with hypertention of 180/120 mm Hg. The serum kalium was 2.7-3.0 mmol/L. In decubitus and standing position, PRA was 8.1 and 9.2μg·L^-1·h^-1, ATH was 198.3 and 279. 1 ng/L, ALD was 285.3 and 761.7 pmol/L, respectively. Dynamic enhanced CT scanning showed a tumor with the diameter of 1.2 cm in right kidney. Case 3 was female, 26 years old. She presented with hypertention of 210/120 mm Hg. The serum kalium was 4. 1 mmol/L. In decubitus and standing position, PRA was 0.1 and 0.3 μg·L^-1·h^-1 , AT]] 56.2 and 71.5 ng/L, ALD 321.3 and 421.1 pmol/L, respectively. On dynamic enhanced CT scanning, a tumor with a diameter of 3.0cm was located in left kidney. Results Partial nephrectomy was successfully performed in 3 patients, including 1 （case 2） retroperitoneal laparoscopic surgery. Pathologic examination revealed encapsulated tumors in all cases. Light microscopically, the tumor consisted of clusters of polygonal cells, and the cell had centrally located nuclei and slightly eosinophilic cytoplasm. Thick walled vessels were usually present. The tumors showed positive immunostaining for actin and CD34. Three patients were followed up for 23,4, 26 months respectively and all remained normotensive without any treatment. No recurrence or metastasis occured. Conclusions Hypertention, increased PRA, secondary aldosteronism, hypokalemia are characteristics for juxtaglomerular cell tumor of the kidney. Dynamic enhanced CT scanning has high sensitivity. Partial nephrectomy or enucleation of tumor are both effective surgical treatment. Retroperitoneal laparoscopic surgery is safe and effective as well.