Juxtaglomerular cell tumor

Pathophysiology

By hypersecretion of renin, JCT causes hypertension, often severe and usually sustained but occasionally paroxysmal, and secondary hyperaldosteronism inducing hypokalemia, though the later can be mild despite high renin. Both of these conditions may be corrected by surgical removal of the tumor.{{cite journal

Histopathology

JCT is morphologically characterized by multiple foci malignant mesenchymal epithelioid cells with, often with admixed necrosis, and a perivascular growth pattern. The immunophenotype is rather characteristic, as the neoplastic cells express renin, CD34, smooth muscle actin, CD138, vimentin, collagen IV and is negative for cytokeratins as well as for S100, c-Kit and desmin.

Diagnosis

Clinically, hypertension, especially when severe or poorly controlled, combined with evidence of a kidney tumor via imaging or gross examination suggest a JCT. Other kidney tumors can cause hypertension by secreting renin. JCTs have a variable appearance and have often being misdiagnosed as renal cell carcinomas; dynamic computed tomography is helpful in the differential diagnosis.

Post-operatively, the presence of renin granules in pathology specimens as well as immunohistochemical analyses could help differentiating this tumor from other primary renal tumors such as hemangiopericytoma, glomus tumor, metanephric adenoma, epithelioid angiomyolipoma, Wilms tumor, solitary fibrous tumor, and some epithelial neoplasms.{{cite journal | doi-access = free

Prognosis

JCT is often described as benign and one case of metastasis has been reported, so its malignant potential is uncertain. In most cases the tumor is encapsulated.{{cite journal

History

Juxtaglomerular cell tumor was first described in 1967 in a paper by Robertson et al., and first named by Kihara et al. in 1968. Since then, approximately 100 case reports have been published. Karyotyping of a small number of these tumors revealed a common loss of chromosomes 9 and 11.

References

References

1. W. Hanna. (April 2, 1979). "Juxtaglomerular cell tumour (reninoma) with paroxysmal hypertension". Can Med Assoc J.
2. Beaudoin, J.. (2008). "A Patient With A Juxtaglomerular Cell Tumor With Histological Vascular Invasion". Nature Clinical Practice Nephrology.
3. Kuroda, Naoto. (2011). "Review of juxtaglomerular cell tumor with focus on pathobiological aspect". Diagnostic Pathology.
4. (May 2013). "Juxtaglomerular cell tumor: multicentric synchronous disease associated with paraneoplastic syndrome". J Clin Oncol.
5. Tanabe. (July 2001). "Dynamic computer tomography is useful in the differential diagnosis of juxtaglomerular cell tumor and renal cell carcinoma.Tanab". Hypertens. Res..