Juxtaglomerular cell tumor

Changed by Yuranga Weerakkody, 1 Jan 2015

Updates to Synonym Attributes

Updates to Synonym Attributes

Title was changed:
Juxtaglomerular cell tumourtumor

Updates to Synonym Attributes

Updates to Article Attributes

Title was changed:
Juxtaglomerular cell tumortumour
Body was changed:

Juxtaglomerular cell tumortumour (JGCT) is an infrequent renal tumortumour of the juxtaglomerular cells. These cells secrete renin and often cause severe hypertension and hypokalemiahypokalaemia.

Epidemiology

JGCT affects all age groups, but is most common in adolescents and young adults, with peak prevalence in the second and third decades of life. JGCT has a female predominance.

Clinical presentation

Patients with JGCT present with headaches, dizziness, double vision, retinopathy, nausea/vomiting and polyuria and most of these may be attributed to hypertension or hypokalemia.

Pathology

JGCT is often well circumscribed, yellow to gray-tan in colorcolour, with a complete or partial fibrous capsule usually observed. Histologically, it consists of solid sheets of closely packed round to polygonal cells.

Radiographic appearance

Imaging findings are variable.

Ultrasound
  • hypoechoic mass
CT
  • variable density with moderate enhancement during late phase after contrast administration
MRI

Reported signal characteristics include

  • T1: iso-signal intensity
  • T2: high-signal intensity 

Treatment and prognosis

Complete tumortumour resection by radical or partial nephrectomy is the best treatment for JGCT. Anti-hypertensive agents can be used to manage hypertension until definitive therapy is planned.

History and etymology

JGCT was originally described in 1967 by Robertson et al, but first named by Kihara et al. in 1968. Approximately 100 case reports have been published.

Differential diagnosis

On imaging consider other renal tumours such as

  • -<p><strong>Juxtaglomerular cell tumor</strong> (<strong>JGCT</strong>) is an infrequent <a href="/articles/renal-tumours">renal tumor</a> of the juxtaglomerular cells. These cells secrete renin and often cause severe hypertension and hypokalemia.</p><h4>Epidemiology</h4><p>JGCT affects all age groups, but is most common in adolescents and young adults, with peak prevalence in the second and third decades of life. JGCT has a female predominance.</p><h4>Clinical presentation</h4><p>Patients with JGCT present with headaches, dizziness, double vision, retinopathy, nausea/vomiting and polyuria and most of these may be attributed to hypertension or hypokalemia.</p><h4>Pathology</h4><p>JGCT is often well circumscribed, yellow to gray-tan in color, with a complete or partial fibrous capsule usually observed. Histologically, it consists of solid sheets of closely packed round to polygonal cells.</p><h4><strong>Radiographic appearance</strong></h4><p>Imaging findings are variable.</p><h5>Ultrasound</h5><ul><li>hypoechoic mass</li></ul><h5>CT</h5><ul><li>variable density with moderate enhancement during late phase after contrast administration</li></ul><h5>MRI</h5><ul>
  • +<p><strong>Juxtaglomerular cell tumour</strong> (<strong>JGCT</strong>) is an infrequent <a href="/articles/renal-tumours">renal tumour</a> of the juxtaglomerular cells. These cells secrete renin and often cause severe hypertension and hypokalaemia.</p><h4>Epidemiology</h4><p>JGCT affects all age groups, but is most common in adolescents and young adults, with peak prevalence in the second and third decades of life. JGCT has a female predominance.</p><h4>Clinical presentation</h4><p>Patients with JGCT present with headaches, dizziness, double vision, retinopathy, nausea/vomiting and polyuria and most of these may be attributed to hypertension or hypokalemia.</p><h4>Pathology</h4><p>JGCT is often well circumscribed, yellow to gray-tan in colour, with a complete or partial fibrous capsule usually observed. Histologically, it consists of solid sheets of closely packed round to polygonal cells.</p><h4><strong>Radiographic appearance</strong></h4><p>Imaging findings are variable.</p><h5>Ultrasound</h5><ul><li>hypoechoic mass</li></ul><h5>CT</h5><ul><li>variable density with moderate enhancement during late phase after contrast administration</li></ul><h5>MRI</h5><p>Reported signal characteristics include</p><ul>
  • -</ul><h4>Treatment and prognosis</h4><p>Complete tumor resection by radical or partial nephrectomy is the best treatment for JGCT. Anti-hypertensive agents can be used to manage hypertension until definitive therapy is planned.</p><h4>History and etymology</h4><p>JGCT was originally described in 1967 by Robertson et al, but first named by Kihara et al. in 1968. Approximately 100 case reports have been published.</p><h4>Differential diagnosis</h4><ul>
  • -<li><a href="/articles/glomus-tumor">glomus tumor</a></li>
  • -<li><a href="/articles/haemangiopericytoma-1">hemangiopericytoma</a></li>
  • -<li><a href="/articles/metanephric-adenoma-of-kidney">metanephric adenoma</a></li>
  • +</ul><h4>Treatment and prognosis</h4><p>Complete tumour resection by radical or partial nephrectomy is the best treatment for JGCT. Anti-hypertensive agents can be used to manage hypertension until definitive therapy is planned.</p><h4>History and etymology</h4><p>JGCT was originally described in 1967 by <strong>Robertson</strong> et al, but first named by <strong>Kihara</strong> et al. in 1968. Approximately 100 case reports have been published.</p><h4>Differential diagnosis</h4><p>On imaging consider other renal tumours such as</p><ul>
  • +<li>
  • +<a href="/articles/glomus-tumor">glomus tumour</a> - kidney</li>
  • +<li>
  • +<a href="/articles/haemangiopericytoma-1">haemangiopericytoma</a> - kidney</li>
  • +<li>
  • +<a href="/articles/metanephric-adenoma-of-kidney">metanephric adenoma</a> - kidney</li>
  • -<li>collecting duct carcinoma</li>
  • -<li>urothelial carcinoma</li>
  • +<li><a title="collecting duct carcinoma" href="/articles/collecting-duct-carcinoma">collecting duct carcinoma</a></li>
  • +<li><a title="urothelial carcinoma" href="/articles/urothelial-carcinoma">urothelial carcinoma</a></li>

References changed:

  • 3. Kim H, Kim C, Choi Y, Ayala A, Amirikachi M, Ro J. Juxtaglomerular Cell Tumor of Kidney with CD34 and CD117 Immunoreactivity: Report of 5 Cases. Arch Pathol Lab Med. 2006;130(5):707-11. <a href="https://doi.org/10.5858/2006-130-707-JCTOKW">doi:10.5858/2006-130-707-JCTOKW</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/16683889">Pubmed</a>
  • 4. Rubenstein J, Eggener S, Pins M, Rosner K, Chugh S, Campbell S. Juxtaglomerular Apparatus Tumor: A Rare, Surgically Correctable Cause of Hypertension. Rev Urol. 2002;4(4):192-5. <a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1475994">PMC1475994</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/16985680">Pubmed</a>
  • 2. Martin S, Mynderse L, Lager D, Cheville J. Juxtaglomerular Cell Tumor: A Clinicopathologic Study of Four Cases and Review of the Literature. Am J Clin Pathol. 2001;116(6):854-63. <a href="https://doi.org/10.1309/B10J-FKQ5-J7P8-WKU4">doi:10.1309/B10J-FKQ5-J7P8-WKU4</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/11764074">Pubmed</a>
  • 2. Martin SA, Mynderse LA, Lager DJ, Cheville JC: Juxtaglomerular cell tumor. A clinicopathologic study of four cases and review of the literature. Am J Clin Pathol 2001, 116(6):854-863. PubMed Abstract

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