Kidney tumor

Benign adult tumors

Juxtaglomerular cell tumor


Editorial Board Member: Debra L. Zynger, M.D.
Deputy Editor-in-Chief: Maria Tretiakova, M.D., Ph.D.
Alcino Pires Gama, M.D.
Bonnie Choy, M.D.

Last author update: 12 April 2022
Last staff update: 12 April 2022

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PubMed Search: Juxtaglomerular cell tumor[TI] kidney pathology

Alcino Pires Gama, M.D.
Bonnie Choy, M.D.
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Cite this page: Gama AP, Choy B. Juxtaglomerular cell tumor. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumorjgtumor.html. Accessed December 10th, 2022.
Definition / general
  • Rare renin secreting tumor arising from the specialized smooth muscle cells of the glomerular afferent arteriole in the renal juxtaglomerular apparatus
Essential features
  • Occurs mostly in young adults; female predominance
  • Frequently associated with severe and poorly controlled hypertension, hypokalemia and hyperaldosteronism secondary to tumor renin secretion
  • Almost always benign with single case of metastatic disease
  • Sheets of polygonal to spindle cells with well defined cell border and prominent vasculature
  • Tumor cells are positive for renin, vimentin, CD34 and CD117
Terminology
  • Reninoma
ICD coding
  • ICD-O: 8361/0 - juxtaglomerular tumor
Epidemiology
Sites
  • Kidney cortex
Clinical features
Diagnosis
  • Diagnosis is suspected in young patients presenting with severe hypertension and hypokalemia and radiologic findings consistent with renal tumor
  • Clinical workup to rule out other causes of hypertension
Laboratory
  • Elevated serum renin and aldosterone levels
Radiology description
Radiology images

Images hosted on other servers:

CT

MRI

Prognostic factors
Case reports
Treatment
  • Surgical resection: partial or total nephrectomy
Gross description
  • Unilateral and solitary (Am J Clin Pathol 2001;116:854, Diagn Pathol 2011;6:80)
  • Well circumscribed; fully or partially encapsulated
  • Gray to yellow-tan, solid cut surface; may have hemorrhage and be partially cystic
  • Average: 2 - 3 cm (range: 0.2 - 15 cm)
Gross images

Images hosted on other servers:

Well circumscribed, solid cut surface

Encapsulated

Hemorrhage and hemosiderin deposition

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Sean Williamson, M.D. and Matthew Wasco, M.D. (Case #467)

Glomoid appearance

Papillary architecture

Prominent vasculature

Polygonal to spindle cells



Contributed by Alcino Pires Gama, M.D.

Normal juxtaglomerular apparatus

Cytology description
  • Sheets of polygonal cells with indistinct cell borders, scant granular cytoplasm and hyperchromatic nuclei, showing mild or no pleomorphism (Int J Surg Pathol 2011;19:93)
  • Nuclei with evenly distributed chromatin and occasional noticeable nucleoli
  • Background of foamy histiocytes and red blood cells
Electron microscopy description
Electron microscopy images

Contributed by Sean Williamson, M.D. and Matthew Wasco, M.D. (Case #467)

Rhomboid shaped renin protogranules

Molecular / cytogenetics description
Sample pathology report
  • Right kidney, mass, partial nephrectomy:
    • Juxtaglomerular cell tumor, measuring 2.5 cm in greatest dimension (see comment)
    • Surgical margins, negative for tumor
    • Comment: The sections show a well circumscribed tumor composed of sheets of uniform round to polygonal cells with clear to slightly eosinophilic cytoplasm and distinct cell borders. Immunohistochemistry was performed and shows diffuse positivity for renin, CD34 and CD117 in the tumor cells. The overall findings support the diagnosis of juxtaglomerular cell tumor.
Differential diagnosis
Board review style question #1

A 25 year old woman presents with uncontrollable hypertension and subsequent workup found a 2 cm left kidney mass. She underwent a partial resection and sections showed the above histologic features. What is the likely diagnosis?

  1. Glomus tumor
  2. Juxtaglomerular cell tumor
  3. Papillary renal cell carcinoma
  4. Solitary fibrous tumor
Board review style answer #1
B. Juxtaglomerular cell tumor should be included in the differential diagnosis of renal tumors with concurrent hypertension, especially in young patients. Histologically, it is composed by sheets of polygonal to spindle cells with well defined cell border and prominent vasculature.

Comment Here

Reference: Juxtaglomerular cell tumor
Board review style question #2
A 20 year old man presents with severe hypertension, hyperreninemia and hypokalemia. Imaging during workup located a 3 cm mass in the right kidney. He underwent a partial nephrectomy and sections of the tumor show sheets of round to polygonal cells with clear to eosinophilic cytoplasm and well defined cell borders. The tumor cells will be positive for which of the following immunohistochemical stains?

  1. CD34
  2. CD99
  3. PAX8
  4. STAT6
Board review style answer #2
A. CD34. Juxtaglomerular cell tumors are positive for renin, vimentin, CD34 and CD117.

Comment Here

Reference: Juxtaglomerular cell tumor
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