Juxtaglomerular cell tumor of kidney with CD34 and CD117 immunoreactivity: Report of 5 cases
ABSTRACT Juxtaglomerular cell tumor is a rare renal neoplasm. Renin immunohistochemistry and electron microscopic documentation of rhomboid crystals are the primary methods of diagnosing this benign tumor.
In this retrospective study, we evaluated the morphologic, immunohistochemical, and ultrastructural features of 5 cases of juxtaglomerular cell tumor to determine the effectiveness of CD34 and CD117 immunohistochemistry for the diagnosis of this tumor.
We reviewed 5 cases with clinical, histologic, immunohistochemical, and ultrastructural aspects.
Three women and 2 men with a mean age of 37.8 years (range, 16-60 years) were included in this study. All patients presented with severe hypertension. All tumors were well circumscribed and ranged from 1.5 cm to 8.5 cm (mean, 4.4 cm). On light microscopic examination, we found solid sheets and nests of tumor cells with oval-to-round nuclei and eosinophilic cytoplasm. Low-power microscopic examination disclosed a hemangiopericytic vascular pattern. Immunohistochemistry results were as follows: vimentin (positive), renin (weakly positive), smooth muscle actin (focal immunoreactivity), and cytokeratin (negative). All 5 tumors were immunoreactive for CD34 and CD117. Electron microscopy revealed rhomboid crystals in the cytoplasm. Postoperatively, 4 patients were normotensive and 1 patient experienced persistent mild hypertension.
Our findings indicate that immunohistochemistry for CD34 and CD117 are effective at diagnosing juxtaglomerular cell tumor. Juxtaglomerular cell tumor should be considered in the diagnosis of any renal tumors with epithelioid cells and negative initial cytokeratin immunohistochemistry.
- SourceAvailable from: Marc Philippe Pusztaszeri
Human pathology 04/2012; 43(4):616; author reply 616-7. DOI:10.1016/j.humpath.2011.12.009 · 2.81 Impact Factor
- "Although the light microscopy and positivity for smooth muscle actin and vimentin could raise the possibility of JCT, our patient's tumor was negative for CD117. This, along with CD 34, has recently been reported to be an effective tool in diagnosing JCT, especially if immunostain for renin is unavailable   . "
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ABSTRACT: Juxtaglomerular cell tumor (JGCT) is an extremely rare renal neoplasm in the pediatric population. It is considered a benign tumor arising from the juxtaglomerular apparatus of the kidney. JGCT has characteristic clinicopathologic features, but its cytogenetic features are unknown. We report a case of JGCT in an 8-year-old female who presented with severe hypertension, elevated serum renin level, and a well circumscribed tumor in the right kidney. Protogranules of renin was identified in the cytoplasm of the tumor cells by electron microscopic examination. Fluorescence in situ hybridization revealed monosomy of chromosomes X, 6, 9, 11, 15, and 21.Pediatric Blood & Cancer 02/2008; 50(2):406-9. DOI:10.1002/pbc.21048 · 2.56 Impact Factor