Korean J Pediatr.  2019 Apr;62(4):144-147. 10.3345/kjp.2018.06926.

Reninoma: a rare cause of curable hypertension

Affiliations
  • 1Department of Pediatrics, Seoul National University Children's Hospital, Seoul National University College of Medicine, Seoul, Korea. cheonghi@snu.ac.kr
  • 2Department of Pediatrics, Korea Cancer Center Hospital, Seoul, Korea.
  • 3Department of Pediatrics, Hallym University Kangnam Sacred Heart Hospital, Seoul, Korea.
  • 4Department of Pediatrics, St. Vincent’s Hospital, The Catholic University of Korea, Suwon, Korea.
  • 5Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea.
  • 6Research Coordination Center for Rare Diseases, Seoul National University Hospital, Seoul, Korea.
  • 7Department of Pathology, Seoul National University Hospital, Seoul, Korea.
  • 8Kidney Research Institute, Medical Research Center, Seoul National University College of Medicine, Seoul, Korea.

Abstract

The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma. The typical clinical presentation of reninoma includes hypokalemia, metabolic alkalosis, and features secondary to the increased activation of the renin-angiotensin system without renal artery stenosis. We report a case of reninoma in a female adolescent with a typical clinical presentation, in which surgical removal of the tumor completely cured hypertension. We discuss here the clinical features, imaging studies, and immunohistochemical examination of the tumor used to establish the diagnosis of reninoma and for the management of the condition.

Keyword

Renal hypertension; Renin; Juxtaglomerular apparatus; Hypokalemia

MeSH Terms

Adolescent
Alkalosis
Child
Diagnosis
Humans
Hypertension*
Hypertension, Renal
Hypokalemia
Juxtaglomerular Apparatus
Renal Artery Obstruction
Renin
Renin-Angiotensin System
Young Adult
Renin
Full Text Links
  • KJP
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr