Juxtaglomerular cell tumor(report of 2 cases and review of the literature) / 中华泌尿外科杂志

ABSTRACT

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 juxtaglo- merular 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 hyperten- sion of 180/110 mm Hg.The laboratory examinations showed that in decubitus and standing position,the plasma rennin activity(PRA)was 3.2?g - L~(-1)?h~(-1)and 36.5?g?L~(-1)?h~(-1);angiotensinⅡwas 54.3 pg/ml and 183.5 pg/ml;aldosterone was 193.5 prnol/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 followsin decubitus and standing position,PRA was 4.3?g?L~(-1)?h~(-1)and 37.0?g?L~(-1)?h~(-1);angio- teusinⅡwas 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 oper- ative time was 3.0 h and 2.0 h,and the blood loss was 175 ml and 112 ml,respectively.There was no mor- tality or postoperative complication.In 1 or 2 postoperative weeks,Case 1 had blood pressure(BP)of 120/70 mm Hg;in deeubitus and standing position,PRA was 1.5?g ~ L~(-1)?b~(-1)and 12.8?g?L~(-1)?h~(-1);angio- tensinⅡwas 30.6 pg/ml and 97.5 pg/ml;aldosterone was 78.5 pmol/L and 192.2 pmol/L,respectively ;se- rum kalium was 4.2 mmol/L.Case 2 had BP of 125/75 mm Hg;in decubitus and standing position,PRA was 1.6?g.L~(-1)?h~(-1)and 12.3?g.L~(-1)?h(-1);angiotensinⅡwas 34.3 pg/ml and 83.5 pg/ml;aldoste- rone 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,CD_(34)expression,and negative MSA,EMA,Bcl-2,?-SmA,AG/AG3,34?En, CD_(117),CD_(31),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 pres- ence of secretion granules and rhomboid-shaped,crystal-like structures in the dilated cisternae of rough endo- plasmic 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.Conelusions Juxtaglomerular cell tumor is a rare tumor which can produce renin.It is characterized by severe hypertension and low serum potassium.La- boratory examination results are helpful for the diagnosisPRA and angiotensinⅡincrease obviously ;aldoste- rone 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 examina- tions.Laparoscopie operation is the first choice of surgical treatment.