Juxtaglomerular cell tumour

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.

General

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

Clinical:

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

Microscopic

Features:

• Polygonal cells.
• Abundant granular, eosinophilic cytoplasm.
• Perinuclear halo.

DDx:

• Metanephric adenoma.
• Papillary RCC.
• Epithelioid angiomyolipoma.
• Chromophobe renal cell carcinoma, eosinophilic variant.^{[citation needed]}

Note - some similarities with:

• Solitary fibrous tumour.
• Glomus tumour.

Images

• Reninoma (nature.com).
• JCT (nih.gov).
• JCT (nih.gov).

Stains

Cytoplasmic granules.

• PAS +ve.
• PASD +ve.

IHC

Features:

• CD34 +ve.
• Vimentin +ve.
• Actin +ve.
• HMB-45 -ve.

Others:

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

Note:

• ‡ Negative keratin staining reported by Chao et al..

EM

Features:

• Vesicles - contain renin.

Molecular

• NOTCH1 rearrangement.

See also

• Kidney tumours.
• Renal tumours with eosinophilic cytoplasm.