Juxtaglomerular Cell Tumor

Martin, Scott A.; Mynderse, Lance A.; Lager, Donna J.; Cheville, John C.

doi:10.1309/b10j-fkq5-j7p8-wku4

Cited by 104 publications

(37 citation statements)

References 58 publications

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“…Other reported symptoms are nausea, polyuria with polydipsia, and fatigue (11). As symptoms are non-specific, there is often a delay in diagnosis of hypertension (12). …”

Section: Discussionmentioning

confidence: 99%

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Reninoma: An Uncommon Cause of Renin-Mediated Hypertension

et al. 2014

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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 magnetic resonance imaging 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.

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“…Other reported symptoms are nausea, polyuria with polydipsia, and fatigue (11). As symptoms are non-specific, there is often a delay in diagnosis of hypertension (12). …”

Section: Discussionmentioning

confidence: 99%

“…Occasional cases of persistent hypertension following reninoma resection are thought to be either due to other causes of hypertension, or to cardiovascular changes from long-standing hypertension (12, 19, 20). Whether these patients eventually become normotensive is difficult to predict given no reports on long-term follow-up.…”

Section: Discussionmentioning

confidence: 99%

Reninoma: An Uncommon Cause of Renin-Mediated Hypertension

et al. 2014

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“…Renin positivity has been observed in some cases of renal cell cancer and renal oncocytoma [11]. α-smooth muscle actin positivity is a feature consistent with the diagnosis of juxtaglomerular cell tumor, whereas negative immunohistochemical staining for CAM5.2, epithelial membrane antigen, and HMB-45 is consistent with the absence of renal epithelial tumors and an epithelioid angiomyolipoma [8, 9, 10]. …”

Section: Discussionmentioning

confidence: 98%

“…The pathobiological aspects of juxtaglomerular cell tumor have been reviewed [8, 9, 10]. Renin positivity has been observed in some cases of renal cell cancer and renal oncocytoma [11].…”

Section: Discussionmentioning

confidence: 99%

Focal Segmental Glomerulosclerosis Secondary to Juxtaglomerular Cell Tumor during Pregnancy: A Case Report

Ohashi

Kobayashi

Arai

et al. 2014

Case Rep Nephrol Dial

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Juxtaglomerular cell tumor is a rare renal neoplasm. Secondary hypertension with juxtaglomerular cell tumor can be seen in females in their 20s and 30s. We present a case of juxtaglomerular cell tumor during pregnancy. A 32-year-old female was hospitalized for refractory hypertension and nephrotic syndrome in the 23rd gestational week. One year before admission, she had been diagnosed with hypertension; plasma renin activity at that time had been 2.3 ng/ml/h. Her blood pressure was uncontrolled during pregnancy, and proteinuria was detected in the 12th gestational week despite the administration of antihypertensive medications. Laboratory data showed proteinuria, hypokalemia, and hypoalbuminemia. In the 25th gestational week, she underwent surgical termination of the pregnancy because of congestive heart failure and acute renal injury. After the termination of the pregnancy and the delivery of a viable fetus, her hypertension and nephrotic syndrome were found to persist with a high plasma renin activity (13 ng/ml/h). Ultrasonography showed a 5.5-cm left renal cystic mass with a partially solid component at the lower renal pole. The left kidney with the renal mass was excised by laparoscopic nephrectomy. Plasma renin activity normalized the next day, with a decrease in blood pressure to 120-130/80-90 mm Hg; however, proteinuria remained at ≥3.5 g/day. On the basis of histopathological findings, the patient was diagnosed with a juxtaglomerular cell tumor and focal segmental glomerulosclerosis. Juxtaglomerular cell tumor is a rare renin-secreting tumor associated with refractory hypertension in young females and is a possible cause of hypertension during pregnancy.

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“…10 Immunohistochemistry will distinguish juxtaglomerular cell tumors from other renal tumours. 2,11 Juxtaglomerular cell tumors stain positive for CD34, which is negative in haemangiopericytoma. Renal cell carcinomas stain positive for cytokeratin, and angiomyolipomas stain positive for HMB-45, both of these are negative in juxtaglomerular cell tumors.…”

Section: Discussionmentioning

confidence: 99%

Ward Round-A rare tumor of the kidney resulting in hypertension, renal failure and a cerebrovascular accident in a young female

Broadis¹,

Ntoto²,

Kamiza³

et al. 2011

Mal. Med. J

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A 17-year-old girl was admitted to our hospital with severe refractory hypertension evolving over approximately 4 years. Despite not having the resources to identify plasma-renin levels and using standard imaging techniques, a juxtaglomerular cell tumor was suspected and was histologically confirmed after surgical excision. This is a potentially lethal condition if left untreated and surgical excision is curative. The benign nature of the tumor is emphasized and its chemical, radiological and microscopic appearance discussed according to the literature. To the best of our knowledge, this is the first reported case of a patient surviving a cerebrovascular accident associated with a juxtaglomerular cell tumor. Case ReportA 17-year-old female was referred to our hospital with a fouryear history of hypertension. Initially this was associated with headaches but six months prior to referral she had suffered a cerebrovascular accident leading to a right hemiplegia. Her blood pressure was poorly controlled, despite being on Furosemide 40mg once daily, Losartan 50mg once daily, Methyldopa 500mg three times daily and Captopril 12.5mg three times daily. She had no known drug allergies and there was nothing else specific in her medical or family history. On examination her heart rate was 80 beats per minute and her blood pressure was 220/160. Her heart sounds were normal and her chest was clear. Her abdomen was soft and non-tender with no organomegaly or palpable masses. Initial investigations included full blood count, urea and electrolytes, twice daily blood pressure monitoring and an ultrasound scan of her abdomen. The results showed she was in renal failure with a urea of 74 mg/dL and creatinine of 5

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Juxtaglomerular Cell Tumor

Cited by 104 publications

References 58 publications

Reninoma: An Uncommon Cause of Renin-Mediated Hypertension

Reninoma: An Uncommon Cause of Renin-Mediated Hypertension

Focal Segmental Glomerulosclerosis Secondary to Juxtaglomerular Cell Tumor during Pregnancy: A Case Report

Ward Round-A rare tumor of the kidney resulting in hypertension, renal failure and a cerebrovascular accident in a young female

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