Kidney tumor
Benign adult tumors
Juxtaglomerular cell tumor
Author: Mandolin S. Ziadie, M.D.
Topic Completed: 1 January 2012
Minor changes: 20 September 2019
Copyright: 2003-2019, 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 answer #1Cite this page: Ziadie MS. Juxtaglomerular cell tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumorjgtumor.html. Accessed September 18th, 2020.
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
- Females > males
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:
Renin granules are PAS+ diastase resistant
Round or oval cells with abundant cytoplasm and vesicular nuclei and chronic inflammatory cells
cytoplasm
and only mild
pleomorphism
Trabecular growth of
uniform epithelioid cells
in hemangiopericytoma-
like vascular network
Various images
Sheets of cells with
hemangiopericytoma-like
vascular pattern
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
Images hosted on other servers:
Rhomboid shaped renin protogranules
Molecular / cytogenetics description
- -9 and -11 (Hum Pathol 2008;39:459)
Differential diagnosis
- Metanephric adenoma: no papillary structures, no atypia, negative for PAS and renin
- Papillary renal cell carcinoma: marked atypia, foam cells, negative for PAS and renin
Board review style question #1
Which immunohistochemical marker is best for
distinguishing juxtaglomerular cell tumor from renal glomus tumor?
A. CD34
B. Smooth muscle actin
C. Renin
D. Vimentin
A. CD34
B. Smooth muscle actin
C. Renin
D. Vimentin
Board review 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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