Kidney tumor
Benign adult tumors
Juxtaglomerular cell tumor


Topic Completed: 1 January 2012

Revised: 20 September 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: "Juxtaglomerular cell tumor"

Mandolin S. Ziadie, M.D.
Page views in 2018: 1,904
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Cite this page: Ziadie MS. Juxtaglomerular cell tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneytumorjgtumor.html. Accessed December 10th, 2019.
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
Treatment
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

Cells have granular
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
Differential diagnosis
Board review 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
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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