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Juxtaglomerular Cell Tumor

MedGen UID:: 87219
•Concept ID:: C0334331
•: Neoplastic Process

Synonyms:	Juxtaglomerular Neoplasm; Juxtaglomerular tumor; Juxtaglomerular Tumor; Reninoma
SNOMED CT:	Reninoma (128860008); Juxtaglomerular tumor (128860008); Reninoma (307618001); Juxtaglomerular tumor (307618001)

Definition

A benign, well circumscribed neoplasm arising from the cortex of the kidney. It secrets renin and the patients usually present with severe hypertension and marked hypokalemia. Morphologically, it is characterized by the presence of sheets of polygonal or spindle-shaped neoplastic cells forming a hemangiopericytic pattern. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVJuxtaglomerular Cell Tumor

Neoplasm
- Neoplasm by Special Category
  - Benign neoplasm
    - Benign Genitourinary System Neoplasm
      - Benign urinary system neoplasm
        Kidney benign neoplasm
        Juxtaglomerular Cell Tumor

Professional guidelines

PubMed

Systematic review of ablation techniques for the treatment of malignant or aggressive benign lesions in children.

Gómez FM, Patel PA, Stuart S, Roebuck DJ
Pediatr Radiol 2014 Oct;44(10):1281-9. Epub 2014 May 13 doi: 10.1007/s00247-014-3001-5. PMID: 24821394

Glomus tumor of kidney: differential diagnosis from juxtaglomerular cell tumor.

Pusztaszeri M
Hum Pathol 2012 Apr;43(4):616; author reply 616-7. doi: 10.1016/j.humpath.2011.12.009. PMID: 22405346

The diagnosis and surgical management of juxtaglomerular cell tumor of the kidney.

Dong D, Li H, Yan W, Xu W, Lu L, Zeng Z
J Hypertens 2010 Mar;28(3):628-32. doi: 10.1097/HJH.0b013e328333b056. PMID: 20051908

See all (3)

Recent clinical studies

Etiology

How to identify juxtaglomerular cell tumor by ultrasound: a case series and review of the literature.

Wang L, Li M, Jin S, Ouyang Y, Wang F, Lv K, Li J, Jiang Y, Liu H, Zhu Q
BMC Med Imaging 2024 Feb 16;24(1):46. doi: 10.1186/s12880-024-01220-9. PMID: 38365645 Free PMC Article

Key Renal Neoplasms With a Female Predominance.

Baniak N, Barletta JA, Hirsch MS
Adv Anat Pathol 2021 Jul 1;28(4):228-250. doi: 10.1097/PAP.0000000000000301. PMID: 34009777

Juxtaglomerular cell tumor: Clinical and immunohistochemical features.

Wang F, Shi C, Cui Y, Li C, Tong A
J Clin Hypertens (Greenwich) 2017 Aug;19(8):807-812. Epub 2017 Mar 20 doi: 10.1111/jch.12997. PMID: 28317244 Free PMC Article

A case of juxtaglomerular cell tumor with novel histologic features.

Onder S, Baydar DE
Int J Surg Pathol 2011 Feb;19(1):65-70. Epub 2009 Apr 29 doi: 10.1177/1066896909333416. PMID: 19403544

Hypertension secondary to renin-secreting juxtaglomerular cell tumor: case report and review of 38 cases.

McVicar M, Carman C, Chandra M, Abbi RJ, Teichberg S, Kahn E
Pediatr Nephrol 1993 Aug;7(4):404-12. doi: 10.1007/BF00857553. PMID: 8398651

See all (12)

Diagnosis

Approach to the Patient: Reninoma.

Hayes AG, Stowasser M, Umapathysivam MM, Falhammar H, Torpy DJ
J Clin Endocrinol Metab 2024 Jan 18;109(2):e809-e816. doi: 10.1210/clinem/dgad516. PMID: 37647894 Free PMC Article

A Young Female With Refractory Hypertension.

Nassiri N, Maas M, Fichtenbaum EJ, Aron M
Urology 2020 Jan;135:e1. Epub 2019 Oct 8 doi: 10.1016/j.urology.2019.09.033. PMID: 31604068

Juxtaglomerular Cell Tumor: A Rare, Curable Cause of Hypertension in a Young Patient.

Walasek A, Gupta S, Fine S, Russo P
Urology 2019 Dec;134:42-44. Epub 2019 Jun 21 doi: 10.1016/j.urology.2019.06.009. PMID: 31233811

Renin‑secreting juxtaglomerular cell tumor of the kidney causing severe hypertension and polyuria.

Prejbisz A, Antoniewicz AA, Kabat M, Kuroszczyk J, Stelmachowska-Banaś M, Januszewicz A
Pol Arch Med Wewn 2014;124(4):207-8. Epub 2014 Feb 20 doi: 10.20452/pamw.2194. PMID: 24556856

Hypertension secondary to a renin-producing juxtaglomerular cell tumor.

Warshaw BL, Anand SK, Olson DL, Grushkin CM, Heuser ET, Lieberman E
J Pediatr 1979 Feb;94(2):247-50. doi: 10.1016/s0022-3476(79)80835-5. PMID: 762618

See all (79)

Therapy

Case report: a nonfunctioning juxtaglomerular cell tumor mimicking renal cell carcinoma.

Dong J, Xu W, Ji Z
Medicine (Baltimore) 2020 Sep 4;99(36):e22057. doi: 10.1097/MD.0000000000022057. PMID: 32899070 Free PMC Article

A Young Female With Refractory Hypertension.

Nassiri N, Maas M, Fichtenbaum EJ, Aron M
Urology 2020 Jan;135:e1. Epub 2019 Oct 8 doi: 10.1016/j.urology.2019.09.033. PMID: 31604068

Juxtaglomerular Cell Tumor: A Rare, Curable Cause of Hypertension in a Young Patient.

Walasek A, Gupta S, Fine S, Russo P
Urology 2019 Dec;134:42-44. Epub 2019 Jun 21 doi: 10.1016/j.urology.2019.06.009. PMID: 31233811

Juxtaglomerular cell tumor: multicentric synchronous disease associated with paraneoplastic syndrome.

Cucchiari D, Bertuzzi A, Colombo P, De Sanctis R, Faucher E, Fusco N, Pellegrinelli A, Arosio P, Angelini C
J Clin Oncol 2013 May 10;31(14):e240-2. Epub 2013 Apr 1 doi: 10.1200/JCO.2012.43.5545. PMID: 23547072

Hypertension due to a renin-secreting juxtaglomerular cell tumor.

Beevers DG, Maheshwari MB, Ryan PG, Moss MS, Harding LK
Am J Hypertens 2008 Dec;21(12):1359-61. Epub 2008 Sep 18 doi: 10.1038/ajh.2008.281. PMID: 18802431

See all (8)

Prognosis

Molecular Characterization of Juxtaglomerular Cell Tumors: Evidence of Alterations in MAPK-RAS Pathway.

Lobo J, Canete-Portillo S, Pena MDCR, McKenney JK, Aron M, Massicano F, Wilk BM, Gajapathy M, Brown DM, Baydar DE, Matoso A, Rioux-Leclerq N, Pan CC, Tretiakova MS, Trpkov K, Williamson SR, Rais-Bahrami S, Mackinnon AC, Harada S, Worthey EA, Magi-Galluzzi C
Mod Pathol 2024 Jun;37(6):100492. Epub 2024 Apr 16 doi: 10.1016/j.modpat.2024.100492. PMID: 38614322

Juxtaglomerular cell tumor: Clinicopathologic evaluation in a large series emphasizing its broad histologic spectrum.

Zhou J, Zheng S, Zhang Y, Yu Y, Zhou L, Zhang W, Wang C, Shen Q, Yang X
Pathol Int 2020 Nov;70(11):844-856. Epub 2020 Sep 1 doi: 10.1111/pin.13009. PMID: 32870578

Juxtaglomerular Cell Tumor With Atypical Pathological Features: Report of a Case and Review of Literature.

Hagiya A, Zhou M, Hung A, Aron M
Int J Surg Pathol 2020 Feb;28(1):87-91. Epub 2019 Aug 21 doi: 10.1177/1066896919868773. PMID: 31432719

A 22-year-old woman with hypertension and hypokalemia due to a juxtaglomerular cell tumor.

Ossareh S, Shooshtarizadeh T, Shadpour P
Iran J Kidney Dis 2011 Nov;5(6):425-8. PMID: 22057077

Review of juxtaglomerular cell tumor with focus on pathobiological aspect.

Kuroda N, Gotoda H, Ohe C, Mikami S, Inoue K, Nagashima Y, Petersson F, Alvarado-Cabrero I, Pan CC, Hes O, Michal M, Gatalica Z
Diagn Pathol 2011 Aug 26;6:80. doi: 10.1186/1746-1596-6-80. PMID: 21871063 Free PMC Article

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Clinical prediction guides

Molecular Characterization of Juxtaglomerular Cell Tumors: Evidence of Alterations in MAPK-RAS Pathway.

Juxtaglomerular cell tumor: Clinical and immunohistochemical features.

Wang F, Shi C, Cui Y, Li C, Tong A
J Clin Hypertens (Greenwich) 2017 Aug;19(8):807-812. Epub 2017 Mar 20 doi: 10.1111/jch.12997. PMID: 28317244 Free PMC Article

Review of juxtaglomerular cell tumor with focus on pathobiological aspect.

Juxtaglomerular cell tumor as a rare cause of hypertension in adults.

Chambô JL, Falci Júnior R, Lucon AM
Int Braz J Urol 2004 Mar-Apr;30(2):119-20. doi: 10.1590/s1677-55382004000200006. PMID: 15703093

Anatomy of the juxtaglomerular apparatus.

Barajas L
Am J Physiol 1979 Nov;237(5):F333-43. doi: 10.1152/ajprenal.1979.237.5.F333. PMID: 386808

See all (19)