Humans ; Female ; Hypertension/complications* ; Kidney Neoplasms/pathology* ; Juxtaglomerular Apparatus/pathology*

Humans ; Female ; Hypertension/complications* ; Kidney Neoplasms/pathology* ; Juxtaglomerular Apparatus/pathology*

The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma. The typical clinical presentation of reninoma includes hypokalemia, metabolic alkalosis, and features secondary to the increased activation of the renin-angiotensin system without renal artery stenosis. We report a case of reninoma in a female adolescent with a typical clinical presentation, in which surgical removal of the tumor completely cured hypertension. We discuss here the clinical features, imaging studies, and immunohistochemical examination of the tumor used to establish the diagnosis of reninoma and for the management of the condition.
Adolescent ; Alkalosis ; Child ; Diagnosis ; Humans ; Hypertension ; Hypertension, Renal ; Hypokalemia ; Juxtaglomerular Apparatus ; Renal Artery Obstruction ; Renin ; Renin-Angiotensin System ; Young Adult

Actins ; metabolism ; Adult ; Antigens, CD34 ; metabolism ; Carcinoma, Renal Cell ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Glomus Tumor ; metabolism ; pathology ; Hemangiopericytoma ; metabolism ; pathology ; Humans ; Hypertension ; etiology ; Juxtaglomerular Apparatus ; metabolism ; pathology ; surgery ; ultrastructure ; Kidney Neoplasms ; complications ; metabolism ; pathology ; surgery ; ultrastructure ; Nephrectomy ; Wilms Tumor ; metabolism ; pathology

Actins ; metabolism ; Adult ; Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Hypertension ; etiology ; Juxtaglomerular Apparatus ; pathology ; surgery ; ultrastructure ; Kidney Neoplasms ; complications ; metabolism ; pathology ; surgery ; ultrastructure ; Nephrectomy ; Vimentin ; metabolism

Juxtaglomerular cell tumor is a rare cause of secondary hypertension. Focal segmental glomerulosclerosis (FSGS) is a clinicopathological entity associated with renal insufficiency and proteinuria. The exact diagnosis and proper management are important in both juxtaglomerular cell tumor and FSGS. We experienced a 26-year-old male who complained of a palpable abdomen mass associated with proteinuria and hypertension. Ultimately, he was diagnosed with a juxtaglomerular cell tumor combined with FSGS after nephrectomy. After operation, his hypertension normalized, while his renal function deteriorated.
Abdomen ; Adult ; Glomerulosclerosis, Focal Segmental ; Humans ; Hypertension ; Juxtaglomerular Apparatus ; Male ; Nephrectomy ; Proteinuria ; Renal Insufficiency

OBJECTIVETo examine the expression of uncoupling protein-2 (UCP2) in the granular cell of women undergoing in vitro fertilization (IVF) and explore its role in embryo development.

METHODSIn this prospective study, the levels of UCP2 and reactive oxygen species (ROS) were measured by the chemiluminescence method in the granular cell of 53 women.

RESULTSWomen with low UCP2 level had higher ROS level, suggesting an inverse relationship between them (r=-0.578, P<0.01), and their oocyte development was impaired. Granular cells of elder women exhibited lowered UCP2 expression.

CONCLUSIONThe granular cells increase UCP2 expression to suppress the elevation of intracellular ROS level through a feedback mechanism and therefore protect the oocytes against oxidative stress.

Adult ; Aging ; metabolism ; Animals ; Embryo Transfer ; Embryonic Development ; Female ; Fertilization in Vitro ; Gene Expression Regulation ; Humans ; Infertility, Female ; metabolism ; Ion Channels ; metabolism ; Juxtaglomerular Apparatus ; cytology ; metabolism ; Mitochondrial Proteins ; metabolism ; Reactive Oxygen Species ; metabolism ; Uncoupling Protein 2 ; Young Adult

OBJECTIVETo investigate the clinical characteristics, morphologic and immunohistochemical features, diagnosis, differential diagnosis, histogenesis and prognosis of renal juxtaglomerular cell tumor (JGCT).

METHODSLight microscopic observation; immunohistochemical assay of CK8, E-cadherin/CK7, CD10, Vim, Actin, CD34, S100, HMB45, CD31, Chr, Syn and CD117, EM; and follow-up were done on all 4 surgically treated JGCT patients.

RESULTSAll 4 JGCT were observed in young adult with clinically uncontrolled severe hypertension. Grossly, the tumor was encapsulated and small in size. Microscopically, the tumor cells grew in sheets predominantly, but papillary and onion-like pattern could also be seen. The stroma contained prominent vasculature that consisted of numerous thin-wall vessels clustering around thick-walled vessels. Tumor cells were rather small, polygonal, with slightly eosinophilic cytoplasm and ill-defined cell border. Nuclei were uniform in size but nuclear atypia and mitosis could be seen. Numerous mast cells were scattered among the tumor cells, and tubules were identified in 3 of 4 cases with positive expression of distal tubule marker of E-cadherin/CK7. Tumor cells positively expressed Vim, Actin, calponin, and CD34. All cases presented ultrastructural features of distinct rhomboid-shaped crystal. There was no recurrence or metastasis but hypertension persisted in three during follow-up (mean 37 months) for all 4 JGCT patients.

CONCLUSIONJGCT, originating from the juxtaglomerular cell, has a distinct benign entity, and it is typically found in young adults with severe hypertension. It has a unique morphology and ultrastructure features and positive immunoreactivity to Vim, Actin, calponin and CD34.

Actins ; analysis ; Adolescent ; Adult ; Antigens, CD34 ; analysis ; Calcium-Binding Proteins ; analysis ; Female ; Humans ; Hypertension ; etiology ; Immunohistochemistry ; Juxtaglomerular Apparatus ; chemistry ; pathology ; ultrastructure ; Kidney Neoplasms ; chemistry ; pathology ; ultrastructure ; Male ; Microfilament Proteins ; analysis

OBJECTIVETo study the morphologic characteristics and immunophenotype of juxtaglomerular cell tumor of the kidney (JGCT), with discussion on its diagnostic clues and possible histogenesis.

METHODSThe clinical, pathologic and immunohistochemical features of 5 cases of JGCT were evaluated. In addition, 5 cases of hemangiopericytoma and 5 cases of cutaneous glomus tumor were selected for comparative immunohistochemical analysis.

RESULTSThe JGCT cases came from 4 females and 1 male (mean age at diagnosis = 32 years). All of them manifested symptoms of systemic hypertension. Four of the patients received partial nephrectomy and the remaining patient was treated by radial nephrectomy. All of them were followed up for a period of 4 to 66 months (average = 27 months). There was no evidence of local recurrence or distant metastases. On gross examination, these JGCTs were well-circumscribed and situated in the renal cortex and measured 4.4 cm in greatest dimension on average. Histologically, the tumor was characterized by the following three features: (1) solid sheets of relatively uniform polygonal to round cells with lightly eosinophilic cytoplasm, sometimes containing PAS-positive intracytoplasmic granules; (2) absence of or very scanty mitotic figures; (3) interstitium rich in thin-walled capillaries, associated with focal hyaline change and hemangiopericytoma-like architectural pattern. Under electron microscopy, characteristic rhomboid-shaped renin granules were found in the cytoplasm. All JGCTs were immunoreactive for renin, CD34, actin, and calponin. In contrast, all glomus tumors were negative for renin and all hemangiopericytomas were negative for actin.

CONCLUSIONSJGCT is a rare benign renal neoplasm typically found in young adults and manifests as systemic hypertension. The tumor cells may be originated from modified vascular smooth muscle cells. The identification of renin granules by electron microscopy and demonstration of the characteristic immunophenotype is the key to correct pathologic diagnosis.

Adult ; Antigens, CD34 ; analysis ; Calcium-Binding Proteins ; analysis ; Female ; Humans ; Immunohistochemistry ; Juxtaglomerular Apparatus ; chemistry ; pathology ; ultrastructure ; Keratins ; analysis ; Kidney Neoplasms ; pathology ; Male ; Microfilament Proteins ; Microscopy, Electron ; Middle Aged

Bartter syndrome is a rare disorder characterized by the association of hypokalemic hypochloremic metabolic alkalosis, hyperreninemia, hyperaldosteronemia, short stature and nephrocalcinosis. This disorder presents with hyperplasia of juxtaglomerular apparatus on renal biopsy. We experienced a case of late-onset Bartter syndrome with nephrocalcinosis in a 9-year-old boy, whose chief pictures were muscle weakness, short stature, persistent sterile pyuria and microscopic hematuria. We report this case with a brief review of related literatures.
Alkalosis ; Bartter Syndrome* ; Biopsy ; Child ; Hematuria ; Humans ; Hyperplasia ; Juxtaglomerular Apparatus ; Male ; Muscle Weakness* ; Nephrocalcinosis ; Pyuria