Juxtaglomerular cell tumour

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Juxtaglomerular cell tumour
Diagnosis in short

Synonyms juxtaglomerular tumour, reninoma
LM DDx metanephric adenoma, papillary renal cell carcinoma, epithelioid angiomyolipoma, chromophobe renal cell carcinoma
Site kidney - see kidney tumours

Associated Dx hypertension
Clinical history usu. young adult or adolescent
Signs hypertension
Prevalence very rare
Blood work increased plasma renin
Prognosis benign (???)
Clin. DDx other causes of hypertension
Treatment surgery

Juxtaglomerular cell tumour, abbreviated JCT, is a rare kidney tumour associated with hypertension and typically seen in adolescents or young adults.

It is also known as juxtaglomerular tumour and reninoma.[1]

General

  • Rare.
  • Etiology: increased renin.
  • Typically adolescents or young adults.[2]

Clinical:[1]

  • Hypertension.
  • Increased aldosterone.
    • Causes hypokalemia.
  • Increased plasma renin.

Microscopic

Features:[3]

  • Polygonal cells.
  • Abundant granular, eosinophilic cytoplasm.[4]
  • Perinuclear halo.

DDx:[2]

Images

Stains

Cytoplasmic granules.[4]

  • PAS +ve.
  • PASD +ve.

IHC

Features:

  • CD34 +ve.[2]
  • Vimentin +ve.
  • Actin +ve.
  • HMB-45 -ve.[3]

Others:[2]

  • CD31 -ve.
  • Desmin -ve.
  • S-100 -ve.
  • Chromogranin -ve.
  • Synaptophysin -ve.
  • NSE -ve.
  • CAM5.2 +ve.[2]
  • CK7 +ve.[2]

Note:

  • ‡ Negative keratin staining reported by Chao 'et al..[3]

EM

Features:

  • Vesicles - contain renin.[5]

See also

References

  1. 1.0 1.1 Wong, L.; Hsu, TH.; Perlroth, MG.; Hofmann, LV.; Haynes, CM.; Katznelson, L. (Feb 2008). "Reninoma: case report and literature review.". J Hypertens 26 (2): 368-73. doi:10.1097/HJH.0b013e3282f283f3. PMID 18192852.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Kuroda, N.; Gotoda, H.; Ohe, C.; Mikami, S.; Inoue, K.; Nagashima, Y.; Petersson, F.; Alvarado-Cabrero, I. et al. (2011). "Review of juxtaglomerular cell tumor with focus on pathobiological aspect.". Diagn Pathol 6: 80. doi:10.1186/1746-1596-6-80. PMID 21871063.
  3. 3.0 3.1 3.2 3.3 Chao, CT.; Chang, FC.; Wu, VC.; Chen, JC. (Jan 2011). "Reninoma.". Kidney Int 79 (2): 260. doi:10.1038/ki.2010.445. PMID 21191395.
  4. 4.0 4.1 Hanna, W.; Tepperman, B.; Logan, AG.; Robinette, MA.; Colapinto, R.; Phillips, MJ. (Apr 1979). "Juxtaglomerular cell tumour (reninoma) with paroxysmal hypertension.". Can Med Assoc J 120 (8): 957-9. PMC 1819229. PMID 436071. //www.ncbi.nlm.nih.gov/pmc/articles/PMC1819229/.
  5. URL: http://path.upmc.edu/cases/case29/micro.html. Accessed on: 18 December 2011.