Kidney tumor
Benign (usually) adult tumors
Juxtaglomerular cell tumor

Author: Mandolin Ziadie, M.D.

Revised: 18 April 2018, last major update January 2012

Copyright: (c) 2003-2018,, Inc.

PubMed Search: "Juxtaglomerular cell tumor"

Cite this page: Ziadie, M.S. Juxtaglomerular cell tumor. website. Accessed October 16th, 2018.
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)
  • 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
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

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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

Images hosted on PathOut server:

Organoid pattern

Epithelial cells with
prominent granular cytoplasm

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Renin granules are PAS+ diastase resistant

Round or oval cells with abundant cytoplasm and vesicular nuclei and chronic inflammatory cells

Various images

Cells have granular
and only mild

Trabecular growth of
uniform epithelioid cells
in hemangiopericytoma-
like vascular network

Various images

Sheets of cells with
like vascular pattern

27 year old woman:
Fig 1: 2 cm, solid, tan cortical mass; Fig 2: solid, compact pattern of round / polygonal cells with focal papillary and tubular patterns;
Fig 3: cells have moderate cytoplasm, round central nuclei, little stroma present; Fig 4: EM shows rhomboid, membrane bound renin
protogranules with 6 - 10 nm periodic crystalline array, some with an electron dense zigzag pattern traversing the array

Positive stains
Negative stains
Electron microscopy description
  • Membrane bound rhomboid shaped renin protogranules, secretory granules
Electron microscopy images

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Rhomboid shaped renin protogranules

Molecular / cytogenetics description
Differential diagnosis