The family history was significant for factor V Leiden deficiency in her father and migraines in her mother and two sisters. Chromosomal abnormalities have been reported in cases of reninoma, with monosomy of chromosome 9, 11, and 15, and polysomy of chromosomes 3, 4, 10 13, 17, and 18 3. In this paper, we present the case of reninoma in an adolescent girl emphasizing clinical presentation, diagnostic evaluation, and medical and surgical treatment of this rare tumor. The tumor cells are characterized by uniform non-enlarged nuclei and a cytoplasm shows numerous pleiomorphic rounded, polygonal, and rhomboidal dense deposits, consistent with renin crystals. Patients with reninoma, however, tend to be older than children with renovascular or renal parenchymal disease, and usually have no or minimal past medical complaints.
The tumor cells were uniform with round nuclei and eosinophilic granular cytoplasm Figure 4 B. To preserve as much normal functional nephron mass as possible, nephron-sparing surgery in the form of partial nephrectomy, wedge excision, or even tumor enucleation are all possible alternatives to complete nephrectomy for reninomas. As a consequence of this hormonal activation, patients develop severe hypertension, potassium wasting, and hypokalemia. A year-old girl was referred to our hospital for treatment and investigations of hypertension. Minimal access surgery ensures better magnification, improved postoperative recovery profile, and better cosmetic results compared to traditional open renal surgery. Ideally, the measurement of plasma and urine electrolytes is done before any treatment including intravenous fluid administration is instituted.
Following the confirmation of the renin-producing tumor in the left kidney, surgical resection of the tumor was performed.
Prevalence of hypertension in children is about 2. The patient was placed renunoma the lateral position with left side up. A renin-secreting tumour with severe hypertension and cardiovascular disease: Following partial nephrectomy, the PRA and plasma aldosterone levels declined rapidly and the blood pressure and potassium supplementation requirements normalized. Clinical consequences of this renin-angiotensin-aldosterone system RAAS activation are peripheral vasoconstriction and salt retention leading to systemic hypertension.
Her headaches were initially treated symptomatically due to a positive family history of migraines. Evaluation of retroperitoneoscopic partial nephrectomy with in situ hypothermic perfusion.
Hypertensive crisis in children. Suggest a Research Topic. There is a mild female to male predominance with a ratio 1.
This can be done at multiple levels of the axis by using renin inhibitors aliskirenACEI, angiotensin II receptor blockers ARBor aldosterone antagonists spironolactone, eplerenone. This case demonstrates the utility of both appropriate imaging studies and selective venous catheterization following provocative administration of an ACE-I for diagnosis.
Further imaging eeview were aimed at localizing relort source of increased renin production. Assessment of urinary excretion of electrolytes is useful but requires h collection of urine.
Because of the association of long-standing severe hypertension and hypokalemia, our further work-up focused on the causes of renin-mediated hypertension. Formal renal angiography, though not specifically indicated in cases of reninoma, is often performed in cases of suspected RAS and in cases likely requiring ajd intervention such as balloon angioplasty or pre-operative tumor embolization. In this paper, we describe the case of reninoma in an adolescent girl, who presented with a long history of headaches suggestive of renin-mediated hypertension.
The diagnosis of reninoma should be considered in patients presenting with severe hypertension, hyperreninemia, hyperaldosteronism, and hypokalemia in whom renovascular and renal parenchymal disease have been excluded.
Reninoma: case report and literature review.
Hormonal studies showed normal plasma and urinary catecholamine and cortisol levels, and normal thyroid function. Am J Surg Pathol 28 8: Reninomas are benign tumors with only literrature reported malignant case Patients typically present with a long reninomaa of headaches leading to a diagnosis of severe hypertension that responds well to antihypertensive treatment targeting the renin-angiotensin-aldosterone system.
Chandar J, Zilleruelo G. To confirm excessive production of renin from this tumor, renal vein renin sampling with lateralization was performed.
Abstract Reninoma is a tumor of the renal juxtaglomerular cell apparatus that causes hypertension and hypokalemia via hypersecretion of renin. The Doppler interrogation of the renal vasculature dominates litersture ultrasound US investigation of hypertension, which detracts from a detailed B-mode evaluation of the kidney substance — whether in search of renal scarring or tumor. Since the first report by Robertson et al. Diagnostic imaging is employed to identify the source of excessive renin production.
Examination of the neck revealed no bruit or thyroid gland enlargement.
Reninoma juxtaglomerular cell tumor is a rare cause of renin-mediated hypertension. Similar articles Malignant hypertension due to a large reninoma: Rfninoma designing the optimal approach for our patient and her renin-secreting tumor, we appreciated the likely benign nature of this lesion Juxtaglomerular cell tumor in an 8-year-old girl.
Renal vein renin sampling with lateralization might help to identify the site of excessive renin production.