Juxtaglomerular cell tumor

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Juxtaglomerular cell tumor (JCT, JGCT, also reninoma) is an extremely rare kidney tumour of the juxtaglomerular cells that typically secretes renin. It often causes severe hypertension that is difficult to control, in adults and children, although among causes of secondary hypertension it is rare. It develops most commonly in young adults, but can be diagnosed much later in life. It is generally considered benign, but its malignant potential is uncertain.[1]

History[edit]

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.[2] Karyotyping of a small number of these tumors revealed a common loss of chromosomes 9 and 11.[1]

Pathology[edit]

By hypersecretion of renin, JCT causes hypertension, often severe and usually sustained but occasionally paroxysmal,[3] and secondary hyperaldosteronism inducing hypokalemia, though the later can be mild despite high renin.[4] Both of these conditions may be corrected by surgical removal of the tumor.[5] Asymptomatic cases have been reported.[2]

Diagnosis[edit]

Pre-operatively, hypertension, especially when severe or poorly controlled, combined with evidence of a kidney tumor via imaging or gross examination suggest a JCT. However, 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.[6] Post-operatively, presence of renin granules in pathology specimens differentiates this tumor from renal hemangiopericytoma and other renal tumors.[7]

Prognosis[edit]

JCT often is described as benign, however one case of metastasis has been reported, so its malignant potential is uncertain.[1] In most cases the tumor is encapsulated.[8]

References[edit]

  1. ^ a b c Capovilla M, Couturier J, Molinié V, Amsellem-Ouazana D, Priollet P, Baumert H, Bruneval P, Vieillefond A (March 2008). "Loss of chromosomes 9 and 11 may be recurrent chromosome imbalances in juxtaglomerular cell tumors". Hum. Pathol. 39 (3): 459–62. doi:10.1016/j.humpath.2007.08.010. PMID 18261631. 
  2. ^ a b Naoto Kuroda et al (2011). "Review of juxtaglomerular cell tumor with focus on pathobiological aspect". Diagnostic Pathology 6: 80. doi:10.1186/1746-1596-6-80. PMC 3173291. PMID 21871063. 
  3. ^ W. Hanna et al (April 2, 1979). "Juxtaglomerular cell tumour (reninoma) with paroxysmal hypertension". Can Med Assoc J. 120 (8): 957–9. PMC 1819229. PMID 436071. 
  4. ^ Beaudoin, J.; Périgny M, Têtu B, Lebel M. (2008). "A Patient With A Juxtaglomerular Cell Tumor With Histological Vascular Invasion". Nature Clinical Practice Nephrology (Laval University, Quebec City, QC, Canada.) 4 (8). doi:10.1038/ncpneph0890. PMID 18654602. 
  5. ^ Wong L, Hsu TH, Perlroth MG, Hofmann LV, Haynes CM, Katznelson L (February 2008). "Reninoma: case report and literature review". J. Hypertens. 26 (2): 368–73. doi:10.1097/HJH.0b013e3282f283f3. PMID 18192852. 
  6. ^ Tanabe et al (July 2001). "Dynamic computer tomography is useful in the differential diagnosis of juxtaglomerular cell tumor and renal cell carcinoma.Tanab". Hypertens Res. 
  7. ^ Martin SA, Mynderse LA, Lager DJ, Cheville JC; Martin; Lager; Cheville (December 2001). "Juxtaglomerular cell tumor: a clinicopathologic study of four cases and review of the literature". Am. J. Clin. Pathol. 116 (6): 854–63. doi:10.1309/B10J-FKQ5-J7P8-WKU4. PMID 11764074. 
  8. ^ Abbi RK, McVicar M, Teichberg S, Fish L, Kahn E (1993). "Pathologic characterization of a renin-secreting juxtaglomerular cell tumor in a child and review of the pediatric literature". Pediatr Pathol 13 (4): 443–51. doi:10.3109/15513819309048234. PMID 8372029.