Juxtaglomerular cell tumour
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Juxtaglomerular cell tumour, abbreviated JCT, is a rare kidney tumour associated with hypertension and typically seen in adolescents or young adults.
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 |
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]
- Metanephric adenoma.
- Papillary RCC.
- Epithelioid angiomyolipoma.
- Chromophobe renal cell carcinoma, eosinophilic variant.[citation needed]
Note - some similarities with:
Images
Stains
Cytoplasmic granules.[4]
- PAS +ve.
- PASD +ve.
IHC
Features:
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]
Molecular
- NOTCH1 rearrangement.[6]
See also
References
- ↑ 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.
- ↑ 2.0 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.
- ↑ 3.0 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.
- ↑ 4.0 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. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1819229/.
- ↑ URL: http://path.upmc.edu/cases/case29/micro.html. Accessed on: 18 December 2011.
- ↑ PMID: 37749094