Secondary hypertension due to a juxtaglomerular cell tumor

Nunes, Igor; Santos, Tiago; Tavares, Joana; Correia, Lurdes; Coutinho, João; Nogueira, J. Braz; Carvalho, L.; Soares, José Luís Ducla

doi:10.1016/j.jash.2018.05.005

Cited by 11 publications

(4 citation statements)

References 10 publications

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“…Interestingly, the patient had elevated PRC, but PRA was in the normal range. To date, approximately 170 cases have been reported, and either elevated PRA [ 3 , 8 – 10 ] or elevated PRC [ 11 , 12 ] is recorded in the literature. PRA, which reflects the biological activity of the renin system, is measured by generating angiotensin I (AngI) from endogenous angiotensinogen, followed by measurement by radioimmunoassay of the generated AngI.…”

Section: Discussionmentioning

confidence: 99%

A case of atypical reninoma with mild hypertension and normal plasma renin activity but elevated plasma renin concentration

Wang

Ding

et al. 2022

BMC Endocr Disord

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Background Reninoma is a rare, benign renal neoplasm. Typical clinical features include severe hypertension, secondary hyperaldosteronism, hypokalaemia and metabolic alkalosis caused by the overproduction of renin. Case presentation A 25-year-old lean Chinese woman with no family history of hypertension was hospitalized for stage 1 hypertension that gradually developed over two years. Endocrine investigation showed hyperreninemia without hyperaldosteronism and hypokalaemia. Interestingly, although the patient had an elevated plasma renin concentration (PRC), her plasma renin activity (PRA) was in the normal range. Abdominal contrast-enhanced computed tomography (CT) scanning revealed a solid, low-density, renal cortical mass with delayed enhancement. Selective renal vein sampling (SRVS) was performed, and a lateralization of the renin secretion from the left kidney was found. Enucleation of the tumour led to a rapid remission of hypertension and hyperreninemia. Based on pathological findings, the patient was diagnosed with reninoma. Immunohistochemical staining of the tumour was positive for Renin, CD34, Vimentin, and synaptophysin (Syn) and negative for somatostatin receptor 2 (SSTR2) and chromogranin A (CgA). Conclusions Reninoma can present as mild hypertension without hyperaldosteronism and hypokalaemia. The clinical features of reninoma may depend on the degree of activation of the renin-angiotensin-aldosterone system (RAAS). PRC should be incorporated in the differential diagnosis of secondary hypertension.

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Section: Discussionmentioning

confidence: 99%

A case of atypical reninoma with mild hypertension and normal plasma renin activity but elevated plasma renin concentration

Wang

Ding

et al. 2022

BMC Endocr Disord

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“…However, patients with malignant hypertension often present with progressive hypertension-mediated organ damage, such as advanced retinopathy and renal damage, neither of which were observed in this patient. Finally, the possibility of reninoma should be considered despite the rarity of this disease [ 16 ]. Renal CT with contrast injection has almost 100% sensitivity for the detection of reninoma [ 17 , 18 ], and renal vein sampling also has 50% to 60% sensitivity for detecting renin lateralization [ 18 ].…”

Section: Discussionmentioning

confidence: 99%

Primary Aldosteronism Masked by Accessory Renal Arteries: A Case Report

Yang

Wang

et al. 2022

JCM

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Primary aldosteronism (PA) is the most frequent form of secondary endocrine hypertension, which is characterized by excessive aldosterone secretion and suppressed renin. The currently recommended diagnostic algorithm is very clear, and the plasma aldosterone-to-renin ratio (ARR) is considered the first-line screening test. However, this indicator is influenced by many factors, some of which may cause false-negative results, consequently leading to underdiagnosed PA. Here, we report the rare case of a 38-year-old man who presented with bilateral accessory renal arteries and aldosterone-producing adenoma but had a negative ARR test result.

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“…Accurate identification of SH is very important because many causes are reversible. Hyperactivity of the renin-angiotensin-aldosterone system caused by juxtaglomerular cell tumor (JGCT; also known as reninoma) is one of the possible causes of SH [4]. Co-occurrence of these two rare clinical entities (SCH and JGCT) is extremely rare.…”

Section: Introductionmentioning

confidence: 99%

Spindle Cell Hemangioma and Atypically Localized Juxtaglomerular Cell Tumor in a Patient with Hereditary BRIP1 Mutation: A Case Report

Papež

Štarha

Pavel

et al. 2021

Genes

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Spindle cell hemangioma is a benign vascular tumor typically occurring in the dermis or subcutis of distal extremities as red–brown lesions that can grow in both size and number over time. They can be very painful and potentially disabling. A family history of cancer or previous history may be relevant and must be taken into consideration. Juxtaglomerular cell tumor (reninoma) is an extremely rare cause of secondary hypertension diagnosed mostly among adolescents and young adults. Excessive renin secretion results in secondary hyperaldosteronism. Subsequent hypokalemia and metabolic alkalosis, together with high blood pressure, are clues for clinical diagnosis. Histological examination of the excised tumor leads to a definitive diagnosis. Reninoma is found in subcapsular localization, in most cases as a solitary mass, in imaging studies of kidneys. Exceptionally, it can be located in another part of a kidney. Both spindle cell hemangioma and reninoma are extremely rare tumors in children and adolescents. Herein, the authors present a case report of a patient with hereditary BRCA1 interacting protein C-terminal helicase 1 (BRIP1) mutation, spindle cell hemangioma, and secondary hypertension caused by atypically localized reninoma.

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Secondary hypertension due to a juxtaglomerular cell tumor

Cited by 11 publications

References 10 publications

A case of atypical reninoma with mild hypertension and normal plasma renin activity but elevated plasma renin concentration

A case of atypical reninoma with mild hypertension and normal plasma renin activity but elevated plasma renin concentration

Primary Aldosteronism Masked by Accessory Renal Arteries: A Case Report

Spindle Cell Hemangioma and Atypically Localized Juxtaglomerular Cell Tumor in a Patient with Hereditary BRIP1 Mutation: A Case Report

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