Kidney tumor
Benign adult tumors
Juxtaglomerular cell tumor
Author: Mandolin S. Ziadie, M.D.
Topic Completed: 1 January 2012
Minor changes: 26 February 2021
Copyright: 2003-2021, PathologyOutlines.com, Inc.
PubMed Search: "Juxtaglomerular cell tumor"
Table of Contents
Definition / general | Epidemiology | Clinical features | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Electron microscopy images | Molecular / cytogenetics description | Differential diagnosis | Board review style question #1 | Board review style answer #1Cite this page: Ziadie MS. Juxtaglomerular cell tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumorjgtumor.html. Accessed May 11th, 2021.
Definition / general
- Extremely rare (< 100 cases) tumor that usually presents in young adults and originates from smooth muscle cells in the walls of the glomerular afferent arteriole (juxtaglomerular apparatus)
Epidemiology
- Mean age 23 - 38 years; some cases in children and in older adults
- F > M
Clinical features
- Associated with signs / symptoms of hyperreninism including hypertension, hyperaldosteronism and hypokalemia
- Clinical differential includes other renin producing tumors including renal cell carcinoma, Wilms tumor, mesoblastic nephroma, hepatoblastoma, lung carcinoma, pancreatic adenocarcinoma, ovarian and soft tissue tumors, glioblastoma multiforme
Case reports
- 10 year old boy with severe hypertension (Am J Surg Pathol 1994;18:837)
- 16 year old girl underwent partial nephrectomy for suspected Wilms tumor (COW #467)
- 25 year old woman with high plasma renin activity (Arch Pathol Lab Med 1993;117:1161)
- 27 year old woman (Arch Pathol Lab Med 2004;128:e112)
- 46 year old man with 15 cm tumor that invaded renal vein and metastasized to both lungs (Am J Surg Pathol 2004;28:1098)
- 52 year old man with metastatic tumor (Am J Surg Pathol 2004;28:1098)
- 2 cases with a dominant papillary pattern (Hum Pathol 1993;24:1168)
Treatment
- Nephron sparing surgery is curative
- Blood pressure may normalize after excision (J Hypertens 2008;26:368)
- Usually benign behavior with no / rare recurrences or metastases (Am J Clin Pathol 2001;116:854)
Gross description
- Well circumscribed, encapsulated, solitary with gray / white / yellow solid cut surface (may have focal hemorrhage / small cysts)
- Average 2 - 3 cm but may be 8 cm
- Usually does not invade beyond kidney
Gross images
Images hosted on other servers:
Tumor at hilum
Microscopic (histologic) description
- Variable; usually sheets of homogenous round cells with granular eosinophilic or clear cytoplasm and distinct cell borders
- Other patterns are sheets / cords of spindle cells with irregular borders, papillary structures lined by flat or cuboidal cells with inner polygonal cells and tubules
- Numerous capillaries with hemangiopericytoma-like growth pattern
- Stroma may be scanty or abundant, composed of hyalinized or myxoid tissue
- Focal hemorrhage, mast cells and lymphoplasmacytic inflammation
- Rare / few mitoses, necrosis or pleomorphism
Microscopic (histologic) images
Contributed by Dr. Sean Williamson and Dr. Matthew Wasco, COW #467
Papillary pattern of homogenous round cells with granular eosinophilic or clear cytoplasm and distinct cell borders; prominent vasculature with hemangiocytoma-like growth
Images hosted on other servers:
Growth pattern of solid sheets
Multinuclear cells are focally seen
Vascular invasion
CD34
Positive stains
Negative stains
Electron microscopy description
- Membrane bound rhomboid shaped renin protogranules, secretory granules
Electron microscopy images
Contributed by Dr. Sean Williamson and Dr. Matthew Wasco, COW #467
Rhomboid shaped renin protogranules
Molecular / cytogenetics description
- -9 and -11 (Hum Pathol 2008;39:459)
Differential diagnosis
Board review style question #1
Which immunohistochemical marker is best for
distinguishing juxtaglomerular cell tumor from renal glomus tumor?
- CD34
- Smooth muscle actin
- Renin
- Vimentin
Board review style answer #1
C. Immunohistochemistry for renin is the best marker to distinguish
juxtaglomerular cell tumor from glomus tumor, although a minor degree of renin positivity has
been described in glomus tumor (Hum Pathol 2017;64:106). Although CD34 has been suggested as a helpful marker
of juxtaglomerular cell tumor, some positivity has been reported in glomus tumor as well. KIT (CD117)
has also been noted as a positive marker of juxtaglomerular cell tumor. Smooth muscle actin and
vimentin are nonspecific for resolving this distinction.
Reference: Kidney tumor - Juxtaglomerular cell tumor
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Reference: Kidney tumor - Juxtaglomerular cell tumor
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