肾球旁细胞瘤二例报告并文献复习

Juxtaglomerular cell tumor(report of 2 cases and review of the literature)

目的提高对肾球旁细胞瘤诊断和治疗的认识水平。方法总结2例肾球旁细胞瘤患者临床诊治资料,结合文献讨论其发病特点、影像学特征、治疗、病理和预后。2例均为女性,年龄分别为20岁和36岁。例1入院时血压180/110 mm Hg,实验室检查血浆肾素活性卧位3．2μg·L^(-1)·h^(-1),立位36．5μg·L^(-1)·h^(-1)；血管紧张素Ⅱ卧位54．3 pg/ml,立位183．5 pg/ml；血醛固酮卧位193．5 pmol/L．立位489．4 pmol/L；血钾2．6 mmol/L。例2人院时血压210/120 mm Hg,肾素活性卧位4．3μg．L^(-1)·h^(-1),立位37．0μg·L^(-1)·h^(-1)；血管紧张素Ⅱ卧位55．6pg/ml,立位200．4pg/ml；血醛固酮卧位162．4 pmol/L,立位506．3 pmol/L；血钾3．0mmol/L。2例CT示右肾下极肿瘤,直径分别为3．0cm和3．5cm。结果例1行腹腔镜下。肾部分切除术,例2因合并右肾动脉狭窄且右肾功能严重受损行腹腔镜下右肾切除术。手术时间分别为3．0 h和2．0h,术中出血少,无手术并发症发生。术后例1血压120/70 mm Hg,肾素活性卧位1．5μg·L^(-1)·h^(-1),立位12．8μg·L^(-1)·h^(-1)；血管紧张素Ⅱ卧位30．6 pg/ml,立位97．5 pg/ml；血醛固酮卧位78．5 pmol/L,立位192．2 pmol/L；血钾4．2 mmol/L。例2血压125/75 mm Hg,肾素活性卧位1．6μg·L^(-1)·h^(-1),立位12．3μg·L^(-1)·h^(-1)；血管紧张素Ⅱ卧位34．3 pg/ml,立位83．5 pg/ml；血醛固酮卧位62．6 pmol/L,立位292．5 pmol/L；血钾4．8 mmol/L。术后病理报告：肿瘤包膜完整,光镜下酷似血管外皮瘤,瘤细胞生长活跃,有轻度核异型性；免疫组化分析瘤细胞Vimentin和CD_(34)(+),MSA、EMA、Bcl-2、α-SmA、AG/AG3、34βEn、CD_(117)、CD_(31)、Iv胶原、Ki-G^-(＜2%)均呈(一)；电镜下瘤细胞核及胞质中的细胞器呈现合成活跃的表现,扩大的粗面内质网池和高尔基体囊泡中可见散在或群集的圆形分泌颗粒及菱形或梯形结晶样物。2例术后分别随访14个月和6个月,肾功能正常,B超和CT等影像学检查未见肿瘤复发。结论肾球旁细胞瘤是一种罕见的产生肾素的肾小球球旁肿瘤,临床特征为严重的高血压和低血钾,实验室检查血肾素活性、血管紧张素Ⅱ明显升高,血醛固酮高于正常1～10倍。确诊需依靠临床表现、光镜、免疫组化和电镜检查。

Objective To improve the diagnosis accuracy and treatment quality of juxtaglomerular cell tumor. Methods The clinical data of 2 female patients （20 and 36 years,respectively） with juxtaglomerular cell tumor were presented and discussed in combination with review of the literature,including the onset characteristics,imaging features, treatment, pathology and prognosis. Case 1 presented with hypertension of 180/110 mm Hg. The laboratory examinations showed that in decubitus and standing position, the plasma rennin activity （PRA） was 3.2 μg·L^-1·h^-1 and 36.5 μg·L^-1·h^-1 ;angiotensin Ⅱ was 54.3 pg/ml and 183.5 pg/ml;aldosterone was 193.5 pmol/L and 489.4 pmol/L,respectively; serum kalium was 2.6 mmol/L. Case 2 presented with hypertension of 210/120 mm Hg. The laboratory examination results were as follows ： in decubitus and standing position, PRA was 4.3 μg·L^-1·h^-1. and 37.0 μg·L^-1·h^-1 ; angiotensin Ⅱ was 55.6 pg/ml and 200.4 pg/ml;aldosterone was 162.4 pmol/L and 506.3 pmol/L, respectively; serum kalium was 3.0 mmol/L. On CT scan, both cases had renal tumor, with the diameter of 3.0 cm and 3. 5 cm,respectively. Results Case 1 underwent laparoscopic partial nephrectomy. Case 2 who had artery stricture and severe functional injure of the right kidney underwent laparoscopic right nephrectomy. The operative time was 3.0 h and 2.0 h,and the blood loss was 175 ml and 112 ml,respectively. There was no mortality or postoperative complication. In 1 or 2 postoperative weeks, Case 1 had blood pressure （BP） of 120/70 mm Hg;in decubitus and standing position,PRA was 1.5 μg·L^-1·h^-1 and 12.8 μg·L^-1·h^-1 ;angiotensin II was 30.6 pg/ml and 97.5 pg/ml;aldosterone was 78.5 pmol/L and 192.2 pmol/L, respectively ;serum kalium was 4.2 mmol/L. Case 2 had BP of 125/75 mm Hg; in decubitus and standing position, PRA was 1.6μg·L^-1·h^-1and 12.3 μg·L^-1·h^-1 ;angiotensin II was 34.3 pg/ml and 83.5 pg/ml;aldosterone was 62.6 pmol/L and 292.5 pmol/L, respectively; serum kalium was 4.8 mmol/L. Pathology showed that the juxtaglomerular cell tumor had intact envelop. Light microscopically,the tumor was very much like a hemangiopericytoma, showing active proliferation and nuclear atypia. The immunohistochemical staining showed positive Vimentin, CD34 expression, and negative MSA, EMA, Bcl-2, α-SmA, AG/AG3,34βEn, CO117 , COal , Iv glue, Ki-G （ 〈 2% ） expression. Ultrastructural changes of the nuclei and some organelles in the cytoplasm were observed under electron microscope. The conspicuous ultrastructural feature was the presence of secretion granules and rhomboid-shaped, crystal-like structures in the dilated cisternae of rough endoplasmic reticulum and vesicles of Golgi complex. The follow-up was 14 and 6 months,respectively; the renal function was normal and no tumor recurrence was found. Conclusions Juxtaglomerular cell tumor is a rare tumor which can produce renin. It is characterized by severe hypertension and low serum potassium. Laboratory examination results are helpful for the diagnosis： PRA and angiotensin II increase obviously;aldosterone is 1 - 10 times more than normal; serum kalium is commonly between 2.1 - 3.5 mmol/L. The definite diagnosis depends on clinical presentations, immunohistochemistry, light and electron microscopic examinations. Laparoscopic operation is the first choice of surgical treatment.