1.
An ectopic renin-secreting adrenal corticoadenoma in a child with malignant hypertension.
Kaslow, AM, Riquier-Brison, A, Peti-Peterdi, J, Shillingford, N, HaDuong, J, Venkatramani, R, Gayer, CP
Physiological reports. 2016;(5)
Abstract
A previously healthy 7-year-old male presented with hypertensive emergency, hypokalemia, and elevated plasma renin activity and aldosterone levels. There was no evidence of virilization or cushingoid features. MRI of the abdomen revealed a large (5 × 5 × 3 cm) peripherally enhancing, heterogeneous mass arising from the left adrenal gland. The patient was treated for a suspected pheochromocytoma. However, his blood pressure was not responsive to alpha-blockade. Blood pressure was controlled with a calcium channel blocker and an angiotensin-converting enzyme (ACE) inhibitor. A complete surgical resection of the mass was performed. Postoperatively, his blood pressure normalized and he did not require antihypertensives. On pathological examination, the tumor tissue stained negative for chromogranin and positive for renin. The final diagnosis was renin-secreting adrenal corticoadenoma, an extremely rare adrenal tumor not previously reported in a pediatric patient. Malignant hypertension due to a renin-secreting tumor may need to be distinguished from a pheochromocytoma if alpha-adrenergic blockade is ineffective.
2.
Management of hypertension and heart failure in patients with Addison's disease.
Inder, WJ, Meyer, C, Hunt, PJ
Clinical endocrinology. 2015;(6):789-92
Abstract
Addison's disease may be complicated by hypertension and less commonly by heart failure. We review the pathophysiology of the renin-angiotensin-aldosterone axis in Addison's disease and how this is altered in the setting of hypertension and heart failure. An essential first step in management in both conditions is optimizing glucocorticoid replacement and considering dose reduction if excessive. Following this, if a patient with Addison's disease remains hypertensive, the fludrocortisone dose should be reviewed and reduced if there are clinical and/or biochemical signs of mineralocorticoid excess. In the absence of such signs, where the renin is towards the upper end of the normal range or elevated, an angiotensin II (AII) receptor antagonist or angiotensin converting enzyme (ACE) inhibitor is the treatment of choice, and the fludrocortisone dose should remain unchanged. Dihydropyridine calcium channel blockers are clinically useful as second line agents, but diuretics should be avoided. In the setting of heart failure, there is an increase in total body sodium and water; therefore, it is appropriate to reduce and rarely consider ceasing the fludrocortisone. Loop diuretics may be used, but not aldosterone antagonists such as spironolactone or eplerenone. Standard treatment with ACE inhibitors, or as an alternative, AII receptor antagonists, are appropriate. Measurements of renin are no longer helpful in heart failure to determine the volume status but plasma levels of brain natriuretic peptide (BNP/proBNP) may help guide therapy.
3.
Reninoma: case report and literature review.
Wong, L, Hsu, TH, Perlroth, MG, Hofmann, LV, Haynes, CM, Katznelson, L
Journal of hypertension. 2008;(2):368-73
Abstract
Reninoma is a tumor of the renal juxtaglomerular cell apparatus that causes hypertension and hypokalemia via hypersecretion of renin. We describe a case of reninoma and provide a review of the literature, with a discussion emphasizing the diagnostic evaluation for such patients. The subject had persistent elevation of both plasma renin activity (PRA) and aldosterone. Imaging studies revealed the presence of a lesion in the renal cortex, which was further identified as a renin-producing lesion via selective venous catheterization following administration of an angiotensin-converting enzyme inhibitor (ACE-I). Following partial nephrectomy, the PRA and plasma aldosterone levels declined rapidly and the blood pressure and potassium supplementation requirements normalized. This case demonstrates the utility of both appropriate imaging studies and selective venous catheterization following provocative administration of an ACE-I for diagnosis.
4.
Renin producing neuroendocrine pancreatic carcinoma--a case report and review of the literature.
Langer, P, Bartsch, D, Gerdes, B, Schwetlick, I, Wild, A, Brehm, B, Erley, C, Lamberts, R
Experimental and clinical endocrinology & diabetes : official journal, German Society of Endocrinology [and] German Diabetes Association. 2002;(1):43-9
Abstract
A 44 year old male patient presented with severe hypertension. The diagnostic work-up revealed elevated levels of plasma renin activity (about 10 times the upper limit of normal) in the presence of normal plasma aldosterone levels and serum potassium concentrations. Renovascular disease was excluded by angiography. Selective renal vein sampling did not show any renin gradient. CT-scans of the abdomen demonstrated normal morphology of the kidneys and adrenals but revealed a big mass in the pancreatic corpus and tail with infiltration of the splenic vein and the presence of enlarged local lymph nodes. The endocrine nature of the pancreatic mass was further supported by a positive octreotide scintigraphy scan. Surgical removal of the tumor by left sided pancreatectomy combined with splenectomy resulted in rapid normalization of elevated renin concentrations as well as blood pressure. Histological examination of the tumor tissue revealed the presence of a neuroendocrine pancreatic carcinoma. Highly (x 70) elevated renin levels were detected by radioimmunoassay in the tumor tissue. To our knowledge this is the first renin-producing neuroendocrine pancreatic carcinoma described in the literature. The present paper describes the case in detail and reviews the available literature on clinical symptomatology, diagnosis and treatment of renin-producing tumors.