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Introduction: A reninoma (juxtaglomerular cell tumor) is a rare cause of secondary hypertension that can present with headaches alongside the triad of severe hypertension, hypokalemia, and metabolic alkalosis. Case Presentation: We describe a case of a 15-year-old previously healthy girl who presented with headaches and hypertensive urgency who had severe hypokalemia, moderate hyponatremia, and elevated aldosterone and renin levels. Abdominal ultrasound and MRI with contrast revealed a unilateral mass localized to the right kidney. Despite treatment of her hypertension, she had persistent hyponatremia with clinical euvolemia which was consistent with the paraneoplastic syndrome of inappropriate antidiuretic hormone secretion (SIADH). She underwent radical nephrectomy which normalized her blood pressure and aldosterone and renin values. The pathology findings were consistent with a reninoma with a mitotic rate of 1–2 mitoses per 10 high power fields. Discussion: Hypertension in the pediatric age group requires workup to rule out secondary causes. The classic triad of hypertension, hypokalemia, and metabolic alkalosis warrants assessment for aldosterone-mediated hypertension which can be a result of a renin-producing tumor. Curative approach requires surgical resection of the tumor. Reninomas may rarely manifest with a paraneoplastic phenomenon including SIADH, as seen in our case. Although reninomas are benign tumors, there are also a few reports of malignant transformation and metastases. Features uncommon in reninomas such as mitotic activity warrant long-term surveillance. Established Facts: Hypertension in the pediatric population requires a broad workup to exclude secondary causes. The triad of hypertension, hypokalemia, and metabolic alkalosis should prompt evaluation of aldosterone-mediated hypertension. Reninoma evaluation should include renin and aldosterone levels, renal imaging, and may require localization with selective renal vein sampling. [ABSTRACT FROM AUTHOR]

Renin‐secreting tumours are rare causes of secondary hypertension and hypokalaemia. They are usually surgically curable, hence proper diagnostic work‐up and tumour localisation is essential. In this paper, we present three Swedish patients recently diagnosed with renin secreting tumours, two with reninomas and one with an extrarenal renin‐producing tumour, to illustrate diagnostic challenges. We also discuss the biochemical work‐up, the pros and cons of different imaging techniques (computer tomography [CT], magnetic resonance imaging and [18F]fluorodeoxyglucose‐positron emission tomography‐CT), as well as how renal vein sampling (RVC) may contribute to localisation of the tumour. [ABSTRACT FROM AUTHOR]

A reninoma is a functional tumor of afferent arteriolar juxtaglomerular cells that secretes the enzyme renin, leading to hyperactivation of the renin-angiotensin-aldosterone system. Reninoma is a potentially curable cause of pathological secondary hyperaldosteronism that results in often severe hypertension and hypokalemia. The lack of suppression of plasma renin contrasts sharply with the much more common primary aldosteronism, but diagnosis is often prompted by screening for that condition. The major differential diagnosis of reninoma is renovascular hypertension. Fewer than 200 cases of reninoma have been described. Reninomas have been reported across a broad demographic but have a 2:1 predilection for women, often of childbearing age. Aldosterone receptor blockade, angiotensin-converting enzyme inhibitors, or angiotensin receptor blockers offer effective medical management but are contraindicated in pregnancy, so surgical curative resection is ideal. The current optimal imaging and biochemical workup of reninoma and management approach (ideally, tumor excision with subtotal renal resection) are described. [ABSTRACT FROM AUTHOR]

Abstract 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.

Abstract A 14‐year‐old male castrated domestic medium‐hair cat with diabetes mellitus was evaluated for vomiting, diarrhea, and anorexia. Two weeks before presentation, the cat had been diagnosed with congestive heart failure and started on furosemide. Initial diagnostic testing identified hypokalemia, systemic hypertension, and hypertrophic cardiomyopathy phenotype, and plasma aldosterone concentration was moderately increased. Abdominal ultrasound examination disclosed bilateral adrenomegaly and a right renal mass, and cytology of a needle aspirate of the mass was consistent with malignant neoplasia. The cat was treated with amlodipine and spironolactone. Because of the unusual presentation for hyperaldosteronism, a comprehensive profile of renin‐angiotensin‐aldosterone system (RAAS) peptides was performed. Results from multiple timepoints indicated persistently and markedly increased plasma renin activity and generalized RAAS upregulation. In addition to the lack of adrenal tumor, the markedly increased plasma renin activity was atypical for primary hyperaldosteronism. These clinical findings are suggestive of primary hyperreninism, a condition previously unreported in cats. The concurrent presence of a renal neoplasm suggests the possibility of a renin‐secreting tumor.

Introduction: Growth failure can result from various underlying causes, necessitating a thorough evaluation. Reninoma, a rare renin-secreting tumor, is an uncommon cause of hypertension, especially in paediatric patients, and has not been associated with growth failure until now., Case Presentation: An 11-year-old girl presented with complaints of poor height gain, headaches, increased thirst, and vomiting. The evaluation revealed severe hypertension and hypokalemic alkalosis. Investigations indicated renin-dependent hypertension, and imaging identified a right renal mass. Renal venous sampling confirmed renin production from the tumor. A right open partial nephrectomy was performed, resulting in the normalization of blood pressure and improved growth. Over the following years, she achieved a significant catch-up growth, regaining her original height percentile and the target adult height., Conclusion: This case adds to the limited literature on paediatric reninoma. It highlights a rare presentation of reninoma causing hypertension and growth failure in a child. The growth failure was likely due to hypokalemic alkalosis-induced low insulin-like growth factor 1 (IGF-1) levels, a mechanism not previously documented in paediatric reninoma cases. Surgical excision of the tumor led to normalization of blood pressure and metabolic alkalosis with significant growth recovery., (© 2024 Walter de Gruyter GmbH, Berlin/Boston.)

A 21-year-old female was referred with a suspected juxtaglomerular cell tumour (reninoma) in the superior pole of the left kidney. She underwent renal biopsy and renal vein sampling (RVS) to confirm the diagnosis. Following an uncomplicated laparoscopic partial nephrectomy, antihypertensive medications were ceased. Histopathology confirmed the diagnosis. Reninoma is a rare but reversible cause of secondary hypertension and should be considered along with primary hyperaldosteronism and pheochromocytoma when investigating hypertension in a young person. The subtle appearance of reninoma on imaging can necessitate other investigations to confirm the diagnosis. Definitive localisation is essential to prevent unnecessary loss of nephrons.

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. [ABSTRACT FROM AUTHOR]

A 14‐year‐old male castrated domestic medium‐hair cat with diabetes mellitus was evaluated for vomiting, diarrhea, and anorexia. Two weeks before presentation, the cat had been diagnosed with congestive heart failure and started on furosemide. Initial diagnostic testing identified hypokalemia, systemic hypertension, and hypertrophic cardiomyopathy phenotype, and plasma aldosterone concentration was moderately increased. Abdominal ultrasound examination disclosed bilateral adrenomegaly and a right renal mass, and cytology of a needle aspirate of the mass was consistent with malignant neoplasia. The cat was treated with amlodipine and spironolactone. Because of the unusual presentation for hyperaldosteronism, a comprehensive profile of renin‐angiotensin‐aldosterone system (RAAS) peptides was performed. Results from multiple timepoints indicated persistently and markedly increased plasma renin activity and generalized RAAS upregulation. In addition to the lack of adrenal tumor, the markedly increased plasma renin activity was atypical for primary hyperaldosteronism. These clinical findings are suggestive of primary hyperreninism, a condition previously unreported in cats. The concurrent presence of a renal neoplasm suggests the possibility of a renin‐secreting tumor. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the clinical value of ultrasound in the diagnosis and treatment of reninoma.Methods: We retrospectively analyzed the ultrasound findings of 9 patients with reninoma confirmed by pathology after surgical resection in our hospital between September 2012 and August 2019. All patients underwent conventional preoperative ultrasonography. Three underwent contrast-enhanced ultrasound (CEUS) and 3 with complete endogenetic tumor underwent intraoperative ultrasonography.Results: Of the 9 patients with conventional ultrasound, 7 had renal space-occupying lesions and 2 had missed diagnosis. A hypoechoic or hyperechoic solid mass with regular morphology, clear boundary, capsule, weak echo halo around the mass, incomplete thin strip color blood flow signal around the mass were shown in 7 cases. Color Doppler displayed color flow signal of the incomplete thin strip around the mass and arterial blood supply with an internal thin branch. In 3 patients (including 2 with missing diagnosis by conventional ultrasound) who underwent preoperative contrast-enhanced ultrasound, the reninoma manifested as low enhancement, and the perfusion pattern showed as slow wash-in and slow wash-out compared with normal renal cortex. In 3 patients (including 2 cases of missed diagnosis by conventional ultrasound) with completely endogenic reninoma, intraoperative ultrasound clearly showed the tumor characteristics, and all successfully underwent laparoscopic ultrasound-guided partial nephrectomy.Conclusions: Preoperative conventional ultrasound combined with CEUS, and clinical features, are helpful for the qualitative diagnosis of reninoma. Laparoscopic partial resection is the first-choice treatment for reninoma. Intraoperative ultrasound can provide real-time imaging, accurately evaluate the tumor status, and provide important information for surgeons.

Le réninome, ou tumeur à cellules de l'appareil juxta-glomérulaire, est une tumeur rénale rare, généralement bénigne, sécrétant de la rénine. Nous rapportons ici le cas d'une jeune femme âgée de 18 ans, sans antécédents médicaux notables, qui a été référée aux urgences de notre hôpital pour un œdème papillaire bilatéral. Le bilan ophtalmologique a été fait en ambulatoire en raison de l'apparition progressive d'un flou visuel bilatéral depuis une semaine, associé à des céphalées en casque. L'IRM cérébrale et la ponction lombaire demandées par l'ophtalmologue et le neurologue permettent d'exclure une hypertension intracrânienne. La patiente présente une hypertension artérielle sévère. Le bilan biologique montre une hypokaliémie, une alcalose métabolique compensée et une microalbuminurie. Lors du séjour, apparaît également une insuffisance rénale aiguë AKIN 1. L'ultrason révèle une lésion tissulo-kystique du pôle supérieur rénal droit. L'IRM abdominale confirme cette lésion et évoque un carcinome rénal à cellules claires, sans invasion calicielle ni vasculaire. Le dosage de la rénine plasmatique est supérieur à 500 mUI/L, avec un dosage de l'aldostérone dans les normes. La biopsie rénale est compatible avec une tumeur de l'appareil juxta-glomérulaire. Après un traitement agressif initial, l'hypertension artérielle est bien contrôlée sous spironolactone seule, permettant finalement un sevrage complet des traitements antihypertenseurs. Une néphrectomie partielle par laparoscopie robot-assistée est effectuée. L'analyse de la pièce opératoire confirme le diagnostic de réninome bénin. Le suivi clinique montre une résolution totale des paramètres clinico-biologiques. Reninoma or juxtaglomerular cell tumor is a rare usually benign renal renin secreting tumor. We report the case of an 18-year old woman, without any medical history, investigated in our hospital's emergency department for a bilateral papilledema. Ambulatory ophthalmological investigations were performed because of a newly occurring blurry vision, associated with diffuse headaches. Cerebral mRI and lumbar puncture recommended by the ophthalmologist and neurologist excluded intra-cranial hypertension. The patient presented with severe hypertension. Laboratory values showed hypokalemia, compensated metabolic alkalosis and microalbuminuria. During the hospital stay, she developed AKIN 1 acute renal injury. Ultrasound revealed a tissular cystic lesion of the superior pole of the right kidney. Abdominal mRI confirmed the lesion and raised suspicion for a renal cell carcinoma without calicial or vascular invasion. Plasma renin value was >500 mUI/L with normal values for plasma aldosterone. Renal biopsy diagnosed a juxtaglomerular cell tumor. After an aggressive initial treatment, hypertension remained well controlled with spironolactone only, finally allowing for withdrawal of all antihypertensive medications. Robot-assisted laparoscopic partial nephrectomy was performed. Studies of the operative specimen confirmed the diagnosis of benign reninoma. Clinical follow-up showed complete resolution of clinical and biological parameters. [ABSTRACT FROM AUTHOR]

BackgroundReninoma is a rare renal endocrine tumor that can cause secondary hypertension, characterized by hypertension, hypokalemia, high renin and aldosterone with normal aldosterone renin ratio (ARR), and occurs more in young female. Mainstream treatment option is surgery, but is less suitable for small or deep lesions, which makes ablation a promising alternative.Case presentationTwo young female with typical manifestations of reninoma, including hypertension, hypokalemia, high renin, high aldosterone and normal ARR, were treated successfully with real-time contrast-enhanced ultrasound guided radiofrequency ablation, and contrast-enhanced ultrasound was also performed before and after treatment for diagnosis and postoperative assessment. Afterward, their blood pressure and laboratory tests became normal and remained steady during the follow-up of 32 and 6 months, respectively.ConclusionContrast-enhanced ultrasound guided radiofrequency ablations is a promising alternative for reninoma treatment with comparable safety and efficacy with surgery, and has advantages especially in small or deep lesions.

Background: Reninoma is a rare renal endocrine tumor that can cause secondary hypertension, characterized by hypertension, hypokalemia, high renin and aldosterone with normal aldosterone renin ratio (ARR), and occurs more in young female. Mainstream treatment option is surgery, but is less suitable for small or deep lesions, which makes ablation a promising alternative. Case presentation: Two young female with typical manifestations of reninoma, including hypertension, hypokalemia, high renin, high aldosterone and normal ARR, were treated successfully with real-time contrast-enhanced ultrasound guided radiofrequency ablation, and contrast-enhanced ultrasound was also performed before and after treatment for diagnosis and postoperative assessment. Afterward, their blood pressure and laboratory tests became normal and remained steady during the follow-up of 32 and 6 months, respectively. Conclusion: Contrast-enhanced ultrasound guided radiofrequency ablations is a promising alternative for reninoma treatment with comparable safety and efficacy with surgery, and has advantages especially in small or deep lesions. [ABSTRACT FROM AUTHOR]

Juxtaglomerular cell tumor (JGCT) is a rare tumor. It is a renin-secreting neoplasm that can cause secondary hypertension. It generally affects adolescents and young adults. Patients typically present with chief complaint of headache leading to a diagnosis of severe hypertension. Herein we report a case of JGCT in a 19-year-old Asian female who initially presented with neurological deficits, and was subsequently found to be hypertensive. She had elevated levels of renin and aldosterone with hypokalemia, and responded to renin inhibitors and an aldosterone antagonist. CT scan demonstrated a 2.2 cm left renal tumor. A partial nephrectomy was performed. Histology showed a well-circumscribed tumor consisting of sheets of polygonal to ovoid cells with eosinophilic cytoplasm and indistinct cell borders. The neoplastic cells are positive for CD34 and synaptophysin. CD117 and tryptase highlight the scattered mast cells within the tumor. After surgical removal of the tumor, the patient’s blood pressure is normal. We also reviewed the literature and discussed the differential diagnoses.

Juxtaglomerular cell tumor (JGCT) is a rare renal tumor with a predominantly benign clinical course. It affects young adults, who often present with hypertension, hypokalemia, and hyperaldosteronism. The tumor cells are round to spindle-shaped with occasional mild to moderate atypia, but mitotic figures are usually absent. Surgical resection is the treatment of choice. Typically, the blood pressure and renin levels normalize after removal of the tumor. Rare cases of metastatic and recurrent JGCT have been reported including cases with vascular invasion. These cases typically occur in older adults and present with larger tumor size (9-15 cm). We report a case of JGCT, 5.5 cm in greatest dimension, with atypical pathological features including invasion of the renal vein, lymphovascular invasion, and significant pleomorphism with rhabdoid morphology, along with a brief review of the literature. [ABSTRACT FROM AUTHOR]

Reninoma (juxtaglomerular cell tumor) is a rare cause of renin-mediated hypertension. We reported a 18 year old woman with history of hypertension for 3 years. Laboratory findings showed severe hypokalemia and markedly increased levels of renin and aldosterone. Kidney ultrasonography, abdominal computed tomography and magnetic resonance imaging revealed a small mass in the middle region of the right kidney. The patient underwent nephron-sparing surgery; immunohistochemical results demonstrated typical features of reninoma. Postoperatively, blood pressure and potassium levels were normal at 1 month follow-up. [ABSTRACT FROM AUTHOR]

A reninoma is an uncommon, benign, renin-secreting juxtaglomerular cell tumor that causes secondary hypertension in young patients. This hypertension is treated by tumor resection. Except for increased levels of plasma renin and angiotensin I and II, the other physical and laboratory examinations and electrocardiographs were within normal limits upon admission of a 19-year-old woman with a reninoma. For percutaneous computed tomography-guided radiofrequency ablation, general anesthesia was induced by thiopental sodium and rocuronium bromide and maintained with servoflurane (2-4 vol%) and oxygen. The operation ended uneventfully in hemodynamic stability. However, the patient complained of dizziness while sitting 5 hours after the operation, and hypotension was diagnosed. After aggressive normal saline (1 L) infusion over 30 min, the hypotension was corrected and the patient recovered without any other surgical complications. Here, we report the anesthetic management of a patient who underwent percutaneous computed tomography-guided radiofrequency ablation for reninoma destruction, particularly focusing on postoperative hypotension.

Reninoma is a rare form of secondary hypertension. The present study reported a case of reninoma that coexisted with an adrenal adenoma in a young female with secondary hypertension during pregnancy. The patient, a 31-year-old female exhibiting hypertension for >1 year, developed a mass in the right adrenal gland, which was detected by an ultrasound scan in the 33rd gestational week. In addition, a well-defined solid mass on the left kidney was detected by a magnetic resonance imaging scan1 month subsequent to the termination of the pregnancy. The blood pressure (BP) of the patient did not change subsequent to the laparoscopic right adrenalectomy. The patient subsequently underwent laparoscopic left partial nephrectomy 2 months later. The BP levels demonstrated a significant reduction from 177/115 to 125/80 mmHg in the same day postoperatively, and stabilized to be within the normal range. The BP level of the patient remained within the normal range subsequent to a 6-month follow-up. Pathological analysis revealed reninoma on the left kidney. The present study aimed to provide information for radiologists who may encounter this type of benign tumor in the future, which exhibits elevated BP levels. [ABSTRACT FROM AUTHOR]

Background: Reninoma is an extremely rare renal tumor characterized by excessive renin secretion causing secondary hypertension and hypokalemia. Reninoma is a benign and highly manageable lesion if it is discovered early and removed surgically. Methods: We report six cases of reninoma and provide a literature review on this rare disease, highlighting the diagnostic evaluation and follow-up of each patient. Results and conclusions: Reninoma should be considered in young adults with elevated renin activity and refractory hypertension. Imaging studies and selective venous catheterization are often helpful in identifying the lesion. In most cases of reninoma presenting with renin-mediated hypertension, conservative surgical treatment should be considered to remove the small, superficial lesion. [ABSTRACT FROM AUTHOR]

A juxtaglomerular cell tumor or reninoma is a very rare renin-secreting tumor of the kidney and can be an unusual cause of secondary hypertension. We report a case of recurrence of this uncommon tumor at the hilum of left kidney in an 8-year-old male child.

Reninoma, a renin-secreting tumor of the juxta-glomerular cells of the kidney, is a rare but surgically treatable cause of secondary hypertension in children. We report a case of reninoma presenting as cardiac syncope with long QTc on electrocardiogram due to hypokalemia.

Reninoma (juxtaglomerular cell tumor) is a rare cause of renin-mediated hypertension. Reninomas are usually diagnosed in adolescents and young adults with occasional reports in younger children. Patients typically present with a long history of headaches leading to a diagnosis of severe hypertension that responds well to antihypertensive treatment targeting the renin-angiotensin-aldosterone system. The clue to clinical diagnosis is the presence of hypokalemia and metabolic alkalosis on the first blood sample drawn before any treatment is instituted. Elevated blood levels of renin and aldosterone confirm the clinical suspicion of renin-mediated hypertension. Diagnostic imaging is employed to identify the source of excessive renin production. While renal ultrasound can miss reninoma, contrast CT or MRI of the kidneys are diagnostic modalities of choice leading to the correct diagnosis. Renal vein renin sampling with lateralization might help to identify the site of excessive renin production. Nephron-sparing surgery is curative with maintenance of normal blood pressure after discontinuation of antihypertensive medications in the majority of patients. 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. We also discuss important points in the management of children presenting with renin-mediated hypertension.

Background: Pheochromocytoma and reninoma represent two rare diseases causing hypertension. We here reported a rare case of association between type 2 multiple endocrine neoplasia related bilateral pheochromocytoma and reninoma. Moreover, polymorphism of ACE gene, which is known to be related to an increase of cardiovascular risk, has been found in the same patient. Case presentation: A 24 year old Caucasian man came to our attention for severe hypertension, resistant to anti-hypertensive polytherapy. At the age of twenty he had undergone total thyroidectomy with lymphadenectomy for medullary carcinoma. Genetic testing showed a RET mutation of codon 918 (exon 16) not documented in other family members. During the follow-up, a progressive increase of urinary metanephrines and catecholamines was recorded. Our evaluation confirmed the presence of severe hypertension (220/140 mmHg) and a severe increase of urinary catecholamines and metanephrines. Due to the presence of hypokalemia, other causes of hypertension were researched leading to the discovery of hyperreninemia (236 μUI/ml) with mild hyperaldosteronism, and a mild increase of the renal artery resistance at ultrasound. An abdominal MRI showed multiple adrenal masses and a right kidney nodular lesion of about 2 cm. The patient underwent bilateral adrenalectomy and right nephrectomy, and histology confirmed the presence of bilateral pheochromocytoma and right reninoma. The post-surgery laboratory evaluation showed a rapid reduction of the urinary metanephrines while plasma renin level remained low in spite of the bilateral adrenalectomy without any mineralocorticoid supplementation. To further investigate these unusual feature, we performed genetic testing for the ACE gene, which revealed the presence of ACE I/D polymorphism. Conclusion: This unique report describes the association between two rare causes of hypertension in the same patient. Furthermore, the absence of requirement of mineralocorticoid supplementation in spite of bilateral adrenalectomy, represent an uncommon and interest finding. [ABSTRACT FROM AUTHOR]

Background/Aim: Reninomas are rare juxtaglomerular tumours which can cause severe hypertension and hypokalaemia. Diagnosis can be problematic and these tumours can be difficult to locate on imaging. In this report we aim to demonstrate the value of carefully performed renal vein renin ratios (RVRRs) to assist in locating these tumours. Method/Results: We report on 3 patients diagnosed with reninoma in our unit. The patients were all female, young (17, 16 and 30 years), severely hypertensive and hypokalaemic (2.5, 2.5 and 3.1 mmol/l). Plasma renin activity (PRA) was elevated (31.9, 274 and 175 ng/ml/h), and aldosterone was high-normal (19.9 ng/dl) or elevated (207 and 109.3 ng/dl). Renal artery stenosis was excluded by renal artery Doppler, DTPA scan and angiography. Renal CT detected the lesion in 2 patients, with one lesion visible on pre- and post-contrast CT and the other on post-contrast CT only. RVRRs were performed several weeks after withdrawing interfering medications, maintaining a <40 mmol/day low-sodium diet and maintaining recumbency overnight the night before and during the procedure. Ratios before and after captopril or enalaprilat administration were obtained and lateralised the tumours in all 3 cases (dominant/non-dominant ratios of 2.3, 4.3 and 3.8). All of the patients underwent nephrectomy yielding a typical juxtaglomerular tumour and resulting in cure of hypertension and hypokalaemia. Conclusions: Reninoma should be suspected in young hypertensives (especially females) with significant hypokalaemia and high PRA or direct renin concentration after renovascular hypertension has been excluded. CT imaging and carefully performed RVRRs provide the highest likelihood of locating these tumours. © 2013 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Introduction: The juxtaglomerular cell tumor is a rare cause of hypertension in young patients, which was first described by Robertson in 1967 and coined by Kihara en1968.1 Up to date, fewer than 100 cases have been reported. Clinically these patients have hypertension, hyperaldosteronism and hypokalemia secondary to excessive renin secretion of by juxtaglomerular cells. Case report: We present a case of juxtaglomerular cells tumor, functional producing renin, which was treated with laparoscopic lumpectomy, who evolved properly, referring symptoms characterized by hypertension and hypokalemia. Conclusion: The renal juxtaglomerular cell tumors are a rare pathology, although rarely malignant, this pathology in most cases it is a functional tumor which produces hiperreninemia and secondary hyperaldosteronism causing hypertension difficult to control and hypokalemia, so it should be considered for surgical treatment which becomes curative in almost 90% of cases. [ABSTRACT FROM AUTHOR]

ims: This case report describes juxtaglomerular cell tumor-a rare renin-producing tumor of the kidney, complicating pregnancy. Clinical case: A previously healthy 24-year-old primigravid woman developed hypertension in the 20th week of pregnancy, leading to a miscarriage in the 28th week. However, hypertension continued after the miscarriage. A more complete examination revealed a solid tumor in the lower pole of the right kidney. The patient underwent a partial right nephrectomy. A histological examination and electron microscopy confirmed the diagnosis of JGCT. The patient´s blood pressure was normalized within 2 weeks. Conclusions: JGCT can lead to miscarriage if undiscovered during pregnancy. A kidney ultrasound should be performed on pregnant women with newly detected hypertension. No staining in early phase of contrast CT is the feature that differentiates JGCT from renal cell carcinoma. These tumors are benign, with only one reported exception, and nephron-sparing surgery is preferable. [ABSTRACT FROM AUTHOR]

Juxtaglomerular cell tumors constitute a very small group among the renal neoplasms. Most secrete renin, causing hypertension and hypokalemia due to secondary hyperaldosteronism. They have characteristic pathological features. For the accurate evaluation, diagnosis has to be confirmed by immunohistochemical staining for renin or electron microscopic demonstration of renin protogranules. This report presents a case of renal juxtaglomerular cell tumor in a 63-year-old man with previously unreported histological findings in addition to classic morphology. [ABSTRACT FROM PUBLISHER]

Reninoma is a tumor that secretes excessive renin and is a rare cause of secondary hypertension. We report a case of reninoma with delayed diagnosis in a 33-year-old woman taking an angiotensin receptor blocker. During angiotensin receptor blocker medication, she had exhibited no electrolyte abnormality. The angiotensin receptor blocker was stopped for pregnancy planning purposes, and subsequent hypokalemia was observed. Abdominal computed tomography showed an enhanced round mass in the right kidney. Right partial nephrectomy was performed and the renal mass was removed. Histologic findings confirmed a diagnosis of reninoma. The patient's blood pressure and serum potassium remained normal after surgery. Diagnosis of reninoma might be delayed in patients taking angiotensin receptor blockers because they can mask hypokalemia due to reninoma. [ABSTRACT FROM AUTHOR]

Hypertension due to a reninoma (suspected on the basis of biochemical and clinical features, and exquisite sensitivity of hypertension to angiotensin-converting enzyme [ACE] inhibition, but not visible on imaging) was cured by laparoscopic nephrectomy. Treatment was followed by an uneventful pregnancy. [ABSTRACT FROM AUTHOR]

Background A 51-year-old woman was referred to the Hypertension Clinic of L'Hôtel-Dieu de Québec Hospital, University of Québec Hospital Centre, with hypertension. Her hypertension had been evolving for approximately 30 years and was refractory to maximum doses of four antihypertensive agents. Routine blood testing revealed mild hypokalemia. Investigations Physical examination, urine and blood analyses including measurement of renin and aldosterone levels, echocardiography, fundoscopy, abdominal--pelvis CT scan and histopathology studies. Diagnosis Juxtaglomerular cell tumor with vascular invasion. Management Radical nephrectomy, and follow-up visits to monitor blood pressure and renin levels. [ABSTRACT FROM AUTHOR]

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Reninoma or juxtaglomerular cell tumor is a rare usually benign renal renin secreting tumor. We report the case of an 18-year old woman, without any medical history, investigated in our hospital's emergency department for a bilateral papilledema. Ambulatory ophthalmological investigations were performed because of a newly occurring blurry vision, associated with diffuse headaches. Cerebral mRI and lumbar puncture recommended by the ophthalmologist and neurologist excluded intra-cranial hypertension. The patient presented with severe hypertension. Laboratory values showed hypokalemia, compensated metabolic alkalosis and microalbuminuria. During the hospital stay, she developed AKIN 1 acute renal injury. Ultrasound revealed a tissular cystic lesion of the superior pole of the right kidney. Abdominal mRI confirmed the lesion and raised suspicion for a renal cell carcinoma without calicial or vascular invasion. Plasma renin value was >500 mUI/L with normal values for plasma aldosterone. Renal biopsy diagnosed a juxtaglomerular cell tumor. After an aggressive initial treatment, hypertension remained well controlled with spironolactone only, finally allowing for withdrawal of all antihypertensive medications. Robot-assisted laparoscopic partial nephrectomy was performed. Studies of the operative specimen confirmed the diagnosis of benign reninoma. Clinical follow-up showed complete resolution of clinical and biological parameters., (Copyright © 2021 Société francophone de néphrologie, dialyse et transplantation. Published by Elsevier Masson SAS. All rights reserved.)

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. [ABSTRACT FROM AUTHOR]

A juxtaglomerular cell tumor or reninoma is a very rare renin-secreting tumor of the kidney and can be an unusual cause of secondary hypertension. We report a case of recurrence of this uncommon tumor at the hilum of left kidney in an 8-year-old male child. [ABSTRACT FROM AUTHOR]

A 16-year-old boy suffered from headaches and dizziness for 2 years. He was found to have remarkably elevated blood pressure (BP) of 180/110 mmHg. Laboratory findings showed a low level of serum potassium and markedly increased plasma renin activity. A solid mass at the periphery of the right kidney and double inferior vena cava (IVC) were detected by abdominal computer tomography (CT). Right partial nephrectomy via laparoscopy was performed on the patient. The histologic and electron microscopic findings comfirmed a diagnosis of juxtaglomerlar cell tumor. The patient had no headache or dizziness with normal BP after surgery. [ABSTRACT FROM AUTHOR]

Objetive: Reninoma, or juxtaglomerular cell tumor, is a benign neoplasm which causes severe hypertension with elevated plasma renin activity.1-3 Usually, it is well localized on computed tomography scan or magnetic resonance images, and complete resection of the tumor with renal function preservation is the optimal treatment.2-4 To describe the technique of a transperitoneal nephron-sparing laparoscopic surgery in a pediatric patient.Material and Methods: Describe the technique of a minimally invasive nephron-sparing surgery for resection of a reninoma tumor in a child.Results: In a 14-year-old girl with history of severe high blood pressure, ultrasound detected a hypoechoic lesion in right kidney. Angio computed tomography scan confirmed a 20-mm diameter lesion in the anterior surface of the lower pole of right kidney and also informed a lower pole renal artery. Transperitoneal laparoscopic approach was performed using 4 ports. After mobilization of the colon, Gerota's fascia was opened and the tumor identified. Both polar and principal renal arteries were dissected and secured with vessel loops. The tumor was resected using an ultrasonic energy device. Selective clamping of the lower pole renal artery was performed during 8 minutes. The transected renal surface was closed with two continuous barbed sutures and the tumor was removed using a handmade endobag. Perirenal drainage was left for 4 days, there were no perioperative complications and the patient was discharged 2 days after surgery. Pathology confirmed typical reninoma immunohistochemical features. Blood pressure and plasma renin levels returned to normal. Postsurgical scintigraphy informed 40% differential function of the right kidney.Conclusion: Reninoma is a benign renal tumor and because of its nature and localization, minimally invasive nephron-sparing surgery should be considered. Strategic planning of the surgery based on preoperative images is essential. If vessel clamping is imperative, minimizing ischemia time and/or selective clamping, when possible, help preserve renal function. [ABSTRACT FROM AUTHOR]