Juxtaglomerular cell tumor:a clinicopathological study of 5 cases / 临床与实验病理学杂志

Purpose To study the clinicopathologic features of juxtaglomerular cell tumor ( JGCT) . Methods The microscopic and immunohistochemical features were examined in 5 cases of JGCT, with review of related literatures. Results Four patients were fe-males and 1 was male, average aged 39 years ( range, 26-60 years) . Four patients had hypertension at diagnosis. Postoperative serum renin and aldosterone levels decreased obviously than before the operation in one patient. In one patient tumor had metastasized to liver and bone and died 14 months after operation. Microscopically, the tumors were well circumscribed and at least partially invested by a thick fibrous capsule. Entrapped tubules were identified. Numerous hemangiopericytoma-like vessels were focally present. The tumor tissue was made up of sheets of ovoid to polygonal cells with regular nuclei, distinct cell borders, moderately abundant eosinophilic cy-toplasm. Mast cells and lymphocyte were common. Thick-walled hyalinized blood vessels were obvious. Necrosis and high mitotic ac-tivity were identified in one case. Immunohistochemically, tumor cells were positive for renin, CD34, vimentin, and SMA (focally). Conclusions JGCT is a rare renal neoplasm. Its diagnosis and differential diagnosis must be established on the clinicopathologic fea-tures, immunohistochemical findings and ultrastructural features. Malignant JGCT is very rare. Its criteria are unknown, but malignant JGCT include histological vascular invasion, a large tumor size, relatively advanced patient age or child, necrosis and/or high mitotic activity.

Classification and treatment of juxtaglomerular cell tumor of the kidney / 中华泌尿外科杂志

Objective To study the classification and treatment of juxtaglomerular cell tumor of the kidney. Methods Five cases of juxtaglomerular cell tumors of the kidney were diagnosed and treated surgically in our hospital during the last 4 years. Seven large series of cases report of juxtaglomerular cell tumors have been published in the Pubmed. These series of cases report plus 1 case report of static juxtaglomerular cell tumor were incorporated into a review of 71 cases previously published in English literature. The clinical presentation, laboratory examination and imaging study were summarized. The clinical presentation including blood pressure, the laboratory examinations including kalium, plasma renin activity and aldosterone, and renal venous sampling for renin assay, and the imaging study including ultrasonography, CT, excretory urography, and selective renal angiography were studied retrospectively. Results The 71 cases juxtaglomerular cell tumors could be classified into 3 types, that is, typical type, atypical type and static type. The 57 typical cases had the typical characteristics of hypertension, hyperaldosteronism, and hypokalemia secondary to tumor renin secretion.The 12 atypical cases had hypertension with normal kalium, and the 2 static cases had normal blood pressure and kalium. Typical juxtaglomerular cell tumor of the kidney should be considered in hypertensive patients with secondary aldosteronism. Atypical juxtaglomerular cell tumor of the kidney should be considered in patients with hypertension and renal tumor. Static juxtaglomerular cell tumor of the kidney should be considered in patients with benign renal tumor. Conclusions The classification of typical, atypical and static juxtaglomerular cell tumors depends on blood pressure and serum kalium. As a surgically corrected disease, juxtaglomerular cell tumor of the kidney should be considered in patients with benign renal tumor, and nephron-sparing surgery is the first choice.

Diagnosis and surgical management of juxtaglomernlar cell tumor of the kidney / 中华泌尿外科杂志

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 juxtaglomerular 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. Thelaboratory examination suggested that in decubitus and standing position, the plasma renin activity (PRA) was 2.2 and 3.5 μg · 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, PRAwas8.1 and 9.2 μg·L-1 · h-1, ATⅡ 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 , ATⅡ 56.2 and 71.5 ng/L, ALD 321.3 and 421.1 pmol/L, respec tively. On dynamic enhanced CT scanning, a tumor with a diameter of 3.0era 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 occurred. 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.

Acute Myocardial Infarction Associated with Secondary Hypertension by Renal Juxtaglomerular Cell Tumor

Most patients with hypertension and no definable cause are said to primary or essential hypertension, while other patients with hypertension and specific causes are siad to secondary hypertension. Approximately 5% fo patients with hypertension have specific causes. The patients of secondary hypertension should not be ignored for the reason: The elimination of cause for hypertension can normalize the blood pressure. Juxtaglomerular cell tumor is a rare condition of secondary hypertension, with only approximately 70 cases reported to date. The higher the level of blood pressure, the most likely that various cardiovascular diseases will develop prematurely through acceleration of atherosclerosis. But Acute myocardial infarction in young hypertensives has been reported rarely. We describe the rare case of a young patient with acute myocardial infarction associated with secondary hypertension by renal juxtaglomerular cell tumor.

A Case of Primary Reninism Manifested by Hypertension with Hypokalemia / 대한내분비학회지

Primary reninism is a rare cause of hypertension manifesting along with hypokalemia. A high level of plasma renin activity and a high level of serum aldosterone are the whole markers of primary reninism. Upon making the diagnosis of primary reninism, other more common causes of aldosteronism must be differentiated, such as renovascular hypertension and primary aldosteronism. Primary reninism is commonly caused by juxtaglomerular cell tumor, which is one of the curable causes of hypertension, and this can be successfully treated by conservative surgery. We report here on a case of primary reninism that was caused by juxtaglomerular cell tumor that developed in a 22-year-old female patient. She was recently diagnosed with hypertension and hypokalemia. She had markedly elevated plasma renin activity and an increased serum aldosterone concentration. Computed tomography revealed a mass located in the right kidney and selective renal vein sampling suggested that the mass was secreting an excess of renin. Right nephrectomy was done and her hypertension with hypokalemia was successfully treated. We report here a case of primary reninism that presented with juxtaglomerular cell tumor along with a review of the literature

A Case of Juxtaglomerular Cell Tumor / 대한신장학회잡지

The juxtaglomerular cell tumor is a rare benign tumor which causes surgically correctable hypertension. We report a case of hypertension caused by juxtaglomerular cell tumor in a 17-year old man. He presented with hypokalemia, metabolic alkalosis and hyperreninemic hyperaldosteronism. Renal angiography showed no evidence of renal artery stenosis. Though no mass was suspected in renal angiography, CT scan showed a well demarcated mass, 3 cm in diameter, in the upper portion of left kidney, which was resected and diagnosed to be a juxtaglomerular cell tumor. After resection of the tumor, blood pressure was normalized with resolution of hypokalemia, metabolic alkalosis and hyperreninemic hyperaldosteronism.

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

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 follows:in 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 diagnosis:PRA 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.