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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 understanding of diagnosis and treatment of renal allograft tumor. Methods The clinical data of 2 patients with glomerular mesothelioma were summarized and their characteristics, imaging characteristics, treatment, pathology and prognosis were discussed. Both cases were female and were 20 and 36 years old respectively. The blood pressure was 180/110 mm Hg on admission, and the plasma level of renin in the laboratory was 3.2 μg · L -1 · h -1 and 36.5 μg · L -1, · H ~ (-1); angiotensin Ⅱ lying 54.3 pg / ml, standing 183.5 pg / ml; serum aldosterone lying position 193.5 pmol / L. Standing 489.4 pmol / L; potassium potassium 2.6 mmol / L. Example 2 hospital blood pressure 210/120 mm Hg, renin active position 4.3μg. L -1 h -1, standing 37.0 μg · L -1 h -1; angiotensin Ⅱ lying 55.6 pg / ml. 4pg / ml; blood aldosterone lying position 162.4 pmol / L, standing 506.3 pmol / L; potassium 3.0mmol / L. Two cases of CT showed right subrenal tumors with diameters of 3.0 cm and 3.5 cm, respectively. Results of Example 1 laparoscopic. Partial nephrectomy, 2 cases of right renal artery stenosis and right renal damage due to severe right laparoscopic right nephrectomy. The operative time was 3.0 h and 2.0 h, respectively, with less intraoperative bleeding and no operative complications. After operation, the blood pressure was 120/70 mm Hg and the activity of renin in the supine position was 1.5 μg · L -1 · h -1 and the level was 12.8 μg · L -1 · h ~ ( -1); angiotensin Ⅱ lying 30.6 pg / ml, standing 97.5 pg / ml; urinary aldosterone lying position 78.5 pmol / L, standing 192.2 pmol / L; 2 mmol / L. The blood pressure was 125/75 mm Hg and the activity of renin in the supine position was 1.6 μg · L -1 · h -1 and the level was 12.3 μg · L -1 · h -1 ); Angiotensin II lying 34.3 pg / ml lying, position 83.5 pg / ml; urinary aldosterone lying position 62.6 pmol / L, stand 292.5 pmol / L; potassium 4.8 mmol / L. Postoperative pathology report: The tumor capsule was intact. The tumor cells resembled hemangiopericytoma under light microscope. The tumor cells were active and had mild nuclear atypia. The expression of Vimentin and CD34 +, MSA, EMA, The expressions of Bcl-2, α-SmA, AG / AG3, 34βEn, CD 117, CD 31, Iv collagen and Ki-G ~ - Organelles in the synthesis of active performance, the enlarged rough endoplasmic reticulum pool and Golgi vesicles in the scattered or clustered round secreted particles and diamond or trapezoidal crystal samples. Two cases were followed up for 14 months and 6 months respectively, with normal renal function. There was no recurrence of tumor in imaging examination such as B-mode ultrasonography and CT. Conclusions Glomerular nephropathy is a rare neoplasia of glomerular mesangioma. Its clinical features are severe hypertension and hypokalemia. Laboratory tests of serum renin activity and angiotensin Ⅱ are significantly increased. Serum aldosteronism than normal 1 to 10 times. The diagnosis depends on clinical manifestations, light microscopy, immunohistochemistry and electron microscopy.