|
1
|
Pathirana NSW, Dissanayake P, Pathmanathan S, Sumanatilleke MR, Eranthaka MDU, Herath DA, Samarasinghe TM, Athukorala ADP. Unravelling a mystery of hypokalemic hypertension- a rare case report of a reninoma. BMC Endocr Disord 2024; 24:271. [PMID: 39695641 DOI: 10.1186/s12902-024-01804-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/11/2024] [Accepted: 12/06/2024] [Indexed: 12/20/2024] Open
Abstract
BACKGROUND Reninoma is a rare cause of secondary hypertension, which can be cured with surgery if identified early before any target organ damage occurs. It leads to hypokalaemia and hypertension and typically responds well to treatment with renin-angiotensin-aldosterone system blockers. However, confirmation of the diagnosis and the localisation of this rare culprit lesion can be challenging. CASE PRESENTATION We describe a case of young-onset hypertension in a 19-year-old girl due to a reninoma. She had resistant hypertension with marked hypokalaemia, which required exceedingly high doses of potassium supplements. Biochemical Investigations revealed secondary hyperaldosteronism. Thus, she underwent a renal angiogram to exclude a renovascular cause for her hypertension. While the renal artery anatomy was normal, there was an exophytic renal lesion in the lower pole of the left kidney. Hence, the diagnosis of a reninoma was suspected. She underwent renal vein sampling to confirm the functionality of the detected tumour, but the results were inconclusive. After a multidisciplinary discussion, based on the clinical evidence, the renal lesion was thought to be a reninoma and a partial nephrectomy was done, removing the lesion. Immediately following resection, her blood pressure and potassium normalised without further drug treatment, and the resected lesion was later confirmed to be a reninoma by histopathological examination. CONCLUSION In young people with hypokalemic hypertension, reninoma should be considered when the more common causes are excluded since prompt treatment with excision of the culprit lesion can cure hypertension and prevent associated morbidity and mortality.
Collapse
Affiliation(s)
| | | | | | | | | | - D A Herath
- Faculty of Medicine, University of Moratuwa, Colombo, Sri Lanka
| | | | | |
Collapse
|
|
2
|
Fabian B, Ragnarsson O, Prazic A, Rydén M, Volpe C, Lindgren O. Diagnostic challenges in patients with reninomas and extrarenal renin-producing tumours. Clin Endocrinol (Oxf) 2024; 101:3-9. [PMID: 38696530 DOI: 10.1111/cen.15069] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/01/2024] [Revised: 04/20/2024] [Accepted: 04/22/2024] [Indexed: 05/04/2024]
Abstract
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.
Collapse
Affiliation(s)
- Botond Fabian
- Department of Endocrinology, Skåne University Hospital, Lund, Sweden
| | - Oskar Ragnarsson
- Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden
- Department of Endocrinology, Sahlgrenska University Hospital, Gothenburg, Sweden
- Wallenberg Centre for Molecular and Translational Medicine, University of Gothenburg, Gothenburg, Sweden
| | | | - Mikael Rydén
- Department of Medicine (H7), Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden
| | - Cristina Volpe
- Department of Molecular Medicine and Surgery, Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden
| | - Ola Lindgren
- Department of Endocrinology, Skåne University Hospital, Lund, Sweden
- Department of Clinical Sciences in Lund, Lund University, Lund, Sweden
| |
Collapse
|
|
3
|
Dong H, Zuo Y, An X, Li H, Zheng Z, Chen Y, Zou Y, Jiang X. Clinical features, laboratory findings and treatment of juxtaglomerular cell tumors: a systemic review. Hypertens Res 2024; 47:1380-1390. [PMID: 38438720 DOI: 10.1038/s41440-024-01606-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/15/2023] [Revised: 01/06/2024] [Accepted: 01/17/2024] [Indexed: 03/06/2024]
Abstract
Juxtaglomerular cell tumors (JGCTs) or reninoma are rare kidney tumors leading to secondary hypertension, and the non-specific clinical manifestations bring about challenges to the diagnosis. This study is to summarize the clinical features, laboratory findings, and treatment of JGCTs. The PubMed, EMBASE database, and manual search were utilized to find all cases, and 158 reports containing 261 patients were identified. Data on patients' demographics, clinical features, diagnostic methods, and treatment options were collected and analyzed. JGCTs occurred predominantly in female patients (female to male ratio, 2.1:1). The median age of patients was 25 years (IQR:18-34 years). Hypertension (97.24%) was the cardinal manifestation. Hypokalemia was reported in 78.71% (159/202) of subjects, and normal serum potassium accounted for 20.79% (42/202). In cases with assessed plasma renin activity (PRA) levels, the median PRA was 7.89 times the upper limit of normal (IQR:3.58-14.41), and 3.82% (5/131) of cases in the normal range. Tumors were detected in 97.8% (175/179) computed tomography (CT), 94.7% (72/76) magnetic resonance imaging (MRI), and 81.5% (110/135) ultrasound, respectively. For 250/261 patients undergoing surgical procedures, 89.14% (197/221), 94.94% (150/158), and 100% (131/131) of patients were restored to normal blood pressure, PRA, and serum potassium, respectively. JGCTs are commonly associated with hypertension, hypokalemia, and hyperreninemia, whereas patients with normotension, normokalemia, and PRA should be systematically pursued after drug-elution lasting for 2 weeks. CT and MRI are more sensitive imaging diagnostic methods. The blood pressure and biochemical parameters of most patients returned to normal after surgery.
Collapse
Affiliation(s)
- Hui Dong
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yujie Zuo
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xuanqi An
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Hongwu Li
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Zhihao Zheng
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yang Chen
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yubao Zou
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xiongjing Jiang
- Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Disease, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
| |
Collapse
|
|
4
|
Hayes AG, Stowasser M, Umapathysivam MM, Falhammar H, Torpy DJ. Approach to the Patient: Reninoma. J Clin Endocrinol Metab 2024; 109:e809-e816. [PMID: 37647894 PMCID: PMC10795928 DOI: 10.1210/clinem/dgad516] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/12/2023] [Revised: 08/02/2023] [Accepted: 08/28/2023] [Indexed: 09/01/2023]
Abstract
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.
Collapse
Affiliation(s)
- Annabelle G Hayes
- Endocrine and Metabolic Unit, Royal Adelaide Hospital, Adelaide, South Australia 5000, Australia
- Faculty of Medicine, University of Adelaide, Adelaide, South Autralia 5000, Australia
| | - Michael Stowasser
- Endocrine Hypertension Research Centre, University of Queensland Diamantina Institute, Brisbane, Queensland 4102, Australia
- Endocrine Hypertension Unit, Greenslopes and Princess Alexandra Hospitals, Brisbane, Queensland 4102, Australia
| | - Mahesh M Umapathysivam
- Endocrine and Metabolic Unit, Royal Adelaide Hospital, Adelaide, South Australia 5000, Australia
- Faculty of Medicine, University of Adelaide, Adelaide, South Autralia 5000, Australia
| | - Henrik Falhammar
- Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm SE-171 76, Sweden
- Department of Endocrinology, Karolinska University Hospital, Stockholm SE-171 76, Sweden
| | - David J Torpy
- Endocrine and Metabolic Unit, Royal Adelaide Hospital, Adelaide, South Australia 5000, Australia
- Faculty of Medicine, University of Adelaide, Adelaide, South Autralia 5000, Australia
| |
Collapse
|
|
5
|
Fu X, Deng G, Wang K, Shao C, Xie LP. Pregnancy complicated by juxtaglomerular cell tumor of the kidney: A case report. World J Clin Cases 2023; 11:2541-2548. [PMID: 37123308 PMCID: PMC10130993 DOI: 10.12998/wjcc.v11.i11.2541] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/26/2022] [Revised: 01/27/2023] [Accepted: 03/17/2023] [Indexed: 04/06/2023] Open
Abstract
BACKGROUND Juxtaglomerular cell tumor (JGCT) of the kidney, also known as reninoma, is a rare renal tumor that typically clinically manifests as hypertension, hypokalemia, high renin, and high aldosterone. It is a cause of secondary hypertension. Pregnancy with JGCT is rarer and easily misdiagnosed as pregnancy-induced hypertension, thus affecting treatment.
CASE SUMMARY A 28-year-old woman presented in early pregnancy with hypertension (blood pressure of 229/159 mmHg), nausea, and occasional dizziness and headache. The patient was diagnosed with pregnancy-induced hypertension, and no relief was found after symptomatic treatment; hence, the pregnancy was terminated by artificial abortion. Her blood pressure remained high following termination of pregnancy. Blood tests suggested hypokalemia (2.997 mmol/L), blood aldosterone measured 613 ng/L, and computed tomography urography showed a tumor in the right kidney. Therefore, laparoscopic partial nephrectomy was performed. After surgery, the patient’s blood pressure returned to normal, and blood potassium, aldosterone, and renin normalized. Postoperative pathological examination revealed JGCT. After long-term follow-up, the patient became pregnant again 6 mo after surgery. No hypertension occurred during pregnancy, and the patient delivered a healthy female neonate.
CONCLUSION Patients with pregnancy complicated by JGCT are difficult to diagnose. Herein, we advise surgeons on proper handling of such situations.
Collapse
Affiliation(s)
- Xian Fu
- Department of Urology, Affiliated Hangzhou First People’s Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China
| | - Gang Deng
- Department of Urology, Affiliated Hangzhou First People’s Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China
| | - Kai Wang
- Department of Urology, Affiliated Hangzhou First People’s Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China
| | - Chang Shao
- Department of Pathology, Affiliated Hangzhou First People’s Hospital, Zhejiang University School of Medicine, Hangzhou 310006, Zhejiang Province, China
| | - Li-Ping Xie
- Department of Urology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, Zhejiang Province, China
| |
Collapse
|
|
6
|
Pan ZJ, Zhang ZC, Jiang XZ, Zhao HF, Wang S, Han RY, Tian J. Juxtaglomerular Cell Tumor: A Rare Cause of Hypertension. Urology 2021; 158:3-4. [PMID: 34536412 DOI: 10.1016/j.urology.2021.08.039] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/13/2021] [Revised: 08/17/2021] [Accepted: 08/29/2021] [Indexed: 11/16/2022]
Affiliation(s)
- Zhong-Jian Pan
- Department of Urology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Zhao-Cun Zhang
- Department of Urology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Xian-Zhou Jiang
- Department of Urology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Hai-Feng Zhao
- Department of Urology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Shuo Wang
- Department of Urology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Ruo-Yan Han
- Department of Urology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China
| | - Jun Tian
- Department of Urology, Qilu Hospital, Cheeloo College of Medicine, Shandong University, Jinan, China.
| |
Collapse
|
|
7
|
Zimmermann Y, Tawadros C, Andrejevic-Blant S, Vogel G. [A rare cause of hypertension with hypokalemia: A case of reninoma]. Nephrol Ther 2021; 17:538-542. [PMID: 34334338 DOI: 10.1016/j.nephro.2021.05.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2021] [Revised: 05/17/2021] [Accepted: 05/27/2021] [Indexed: 11/17/2022]
Abstract
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.
Collapse
Affiliation(s)
- Yaël Zimmermann
- Service de néphrologie, hôpital Riviera-Chablais site de Rennaz, route du Vieux Sequoia 20, 1847 Rennaz, Suisse
| | - Cécile Tawadros
- Service d'urologie, hôpital Riviera-Chablais site de Rennaz, 1847 Rennaz, Suisse
| | | | - Gérard Vogel
- Service de néphrologie, hôpital Riviera-Chablais site de Rennaz, route du Vieux Sequoia 20, 1847 Rennaz, Suisse.
| |
Collapse
|
|
8
|
Ye Z, Fan H, Tong A, Xiao Y, Zhang Y. The Small Size and Superficial Location Suggest That Laparoscopic Partial Nephrectomy Is the First Choice for the Treatment of Juxtaglomerular Cell Tumors. Front Endocrinol (Lausanne) 2021; 12:646649. [PMID: 33995279 PMCID: PMC8120284 DOI: 10.3389/fendo.2021.646649] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/27/2020] [Accepted: 04/09/2021] [Indexed: 11/22/2022] Open
Abstract
BACKGROUND Juxtaglomerular cell tumor (JGCT) is a very rare disease, and surgical resection is the only possible way to cure this tumor. Open nephrectomy and partial nephrectomy have been reported to manage JGCTs with excellent results in the previous reviews. Laparoscopic surgery has been popularized in recent years, while critical issues associated with laparoscopic surgical management have been seldom reported. We summarized the JGCTs in our center to discover the optimal surgical management and its anatomic foundation. METHODS In this retrospective study, we enrolled a total of 14 JGCT patients. All patients received surgeries and were followed up for up to 11 years. We mainly summarized the size and location of tumors, imaging features, and surgical strategies. A descriptive statistical analysis was performed. RESULTS The JGCTs in this study had a median size of 1.35 cm and all located superficially, mainly in the cortical or subcortical area of the kidney. All 14 patients had hypertension, ten had hypokalemia, and seven had elevated plasma renin activity. Pathologically, JGCT cells were polygonal or spindle shape, with positive CD34 and vimentin immunostaining. All patients received partial nephrectomy; nine were laparoscopic, and five were open. Laparoscopic partial nephrectomy (LPN) was performed in seven out of eight patients over the last nine years. Postoperative blood pressure, serum potassium, and plasma renin activity were normal in all patients. No recurrence occurred within a median follow-up of 60 months. CONCLUSION The small size and superficial location are the characteristic anatomic features of JGCT; they suggest that LPN is the preferred surgical strategy. Laparoscopic ultrasound is helpful for the intraoperative detection of small JGCTs. Longer follow-up is required to examine the biological behavior of JGCTs and the effect of LPN.
Collapse
Affiliation(s)
- Zixing Ye
- Department of Urology, Peking Union Medical College Hospital, Beijing, China
| | - Hua Fan
- Department of Urology, Peking Union Medical College Hospital, Beijing, China
| | - Anli Tong
- Department of Endocrinology, Peking Union Medical College Hospital, Beijing, China
| | - Yu Xiao
- Department of Pathology, Peking Union Medical College Hospital, Beijing, China
| | - Yushi Zhang
- Department of Urology, Peking Union Medical College Hospital, Beijing, China
- *Correspondence: Yushi Zhang,
| |
Collapse
|
|
9
|
A case of juxtaglomerular cell tumor with an unusual clinical presentation. HUMAN PATHOLOGY: CASE REPORTS 2020. [DOI: 10.1016/j.ehpc.2020.200446] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022] Open
|
|
10
|
Jiang Y, Hou G, Zhu Z, Zang J, Cheng W. Increased FDG Uptake on Juxtaglomerular Cell Tumor in the Left Kidney Mimicking Malignancy. Clin Nucl Med 2020; 45:252-254. [PMID: 31977483 DOI: 10.1097/rlu.0000000000002924] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022]
Abstract
Juxtaglomerular cell tumor is a rare and benign tumor arising from the juxtaglomerular apparatus that overproduces renin, resulting in secondary hypertension. A 29-year-old woman was incidentally found to have a left renal mass by ultrasonography in a routine health examination. Contrast-enhanced CT results suggested renal cell carcinoma. FDG PET/CT performed for metastatic workup showed increased FDG uptake to the left renal mass and did not reveal any other abnormal FDG-avid lesions. The renal mass was surgically resected and pathological examination confirmed the juxtaglomerular cell tumor of the left kidney.
Collapse
Affiliation(s)
| | | | | | - Jinfeng Zang
- Department of Pathology, Peking Union Medical College Hospital Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | | |
Collapse
|
|
11
|
Qiuyang LMD, Ying ZMD, Yong SMD, Aitao GMD, Nan LBS, Yukun LMD, Jie TMD. Clinical Application of Ultrasound in the Diagnosis and Treatment of Reninoma. ADVANCED ULTRASOUND IN DIAGNOSIS AND THERAPY 2020. [DOI: 10.37015/audt.2020.200013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
|
|
12
|
Faucon AL, Bourillon C, Grataloup C, Baron S, Bernadet-Monrozies P, Vidal-Petiot E, Azizi M, Amar L. Usefulness of Magnetic Resonance Imaging in the Diagnosis of Juxtaglomerular Cell Tumors: A Report of 10 Cases and Review of the Literature. Am J Kidney Dis 2019; 73:566-571. [DOI: 10.1053/j.ajkd.2018.09.005] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/10/2018] [Accepted: 09/07/2018] [Indexed: 11/11/2022]
|
|
13
|
Liu K, Wang B, Ma X, Li H, Zhang Y, Li J, Yao Y, Tang L, Xuan Y, Guo A, Zhang X. Minimally Invasive Surgery-Based Multidisciplinary Clinical Management of Reninoma: A Single-Center Study. Med Sci Monit 2019; 25:1600-1610. [PMID: 30822300 PMCID: PMC6407327 DOI: 10.12659/msm.913826] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022] Open
Abstract
Background This article presents our experience in managing a rare kidney tumor – reninoma – by analyzing a relatively large series of cases from a single center. Material/Methods Nine cases of reninoma were reviewed. Clinical manifestations, imaging examinations, laboratory examinations, perioperative data, and pathological findings were summarized. A 58.8-month follow-up was performed to evaluate patient survival and recrudescence. Results The main clinical manifestations were hypertension, hypokalemia, headache, dizziness, nausea, vomiting, palpation, and sweating. Three patients had hypertensive end-organ damage, including brain hemorrhage, gestation termination, and grade III ocular fundus changes. All patients underwent retroperitoneal laparoscopic partial nephrectomy successfully. The mean warm ischemic time was 23.4 min. The median operation time was 95.1 min, with a median estimated blood loss of 60 ml. The median hospital stay was 6 days. No serious intraoperative or postoperative complications occurred. The histology and electron microscopy findings confirmed the diagnosis of reninoma in all cases. After 58.8 months of follow-up, symptoms involving hypertension were relieved in all patients, and no tumor recurrence or metastasis was detected. Conclusions Reninoma may have severe consequences despite being a benign tumor. Retroperitoneal laparoscopic partial nephrectomy is a feasible and effective method for the surgical removal of reninoma. Multidisciplinary cooperation plays an important role in improving the diagnosis and enabling the early surgical treatment of reninoma. Especially in cases of reninoma with moderate and high RENAL scores, an accurate diagnosis of reninoma based on multidisciplinary cooperation facilitates the selection of less invasive surgical approaches.
Collapse
Affiliation(s)
- Kan Liu
- Department of Urology, State Key Laboratory of Kidney Diseases, Chinese People's Liberation Army (PLA) General Hospital/Chinese PLA Medical Academy, Beijing, China (mainland)
| | - Baojun Wang
- Department of Urology, State Key Laboratory of Kidney Diseases, Chinese People's Liberation Army (PLA) General Hospital/Chinese PLA Medical Academy, Beijing, China (mainland)
| | - Xin Ma
- Department of Urology, State Key Laboratory of Kidney Diseases, Chinese People's Liberation Army (PLA) General Hospital/Chinese PLA Medical Academy, Beijing, China (mainland)
| | - Hongzhao Li
- Department of Urology, State Key Laboratory of Kidney Diseases, Chinese People's Liberation Army (PLA) General Hospital/Chinese PLA Medical Academy, Beijing, China (mainland)
| | - Yu Zhang
- Department of Urology, State Key Laboratory of Kidney Diseases, Chinese People's Liberation Army (PLA) General Hospital/Chinese PLA Medical Academy, Beijing, China (mainland)
| | - Jinlong Li
- Department of Pathology, Chinese People's Liberation Army (PLA) General Hospital/Chinese PLA Medical Academy, Beijing, China (mainland)
| | - Yuanxin Yao
- Department of Urology, State Key Laboratory of Kidney Diseases, Chinese People's Liberation Army (PLA) General Hospital/Chinese PLA Medical Academy, Beijing, China (mainland)
| | - Lu Tang
- Department of Urology, State Key Laboratory of Kidney Diseases, Chinese People's Liberation Army (PLA) General Hospital/Chinese PLA Medical Academy, Beijing, China (mainland)
| | - Yundong Xuan
- Department of Urology, State Key Laboratory of Kidney Diseases, Chinese People's Liberation Army (PLA) General Hospital/Chinese PLA Medical Academy, Beijing, China (mainland)
| | - Aitao Guo
- Department of Pathology, State Key Laboratory of Kidney Diseases, Chinese People's Liberation Army (PLA) General Hospital/Chinese PLA Medical Academy, Beijing, China (mainland)
| | - Xu Zhang
- Department of Urology, State Key Laboratory of Kidney Diseases, Chinese People's Liberation Army (PLA) General Hospital/Chinese PLA Medical Academy, Beijing, China (mainland)
| |
Collapse
|
|
14
|
Maiolino G, Battistel M, Barbiero G, Bisogni V, Rossi GP. Cure With Cryoablation of Arterial Hypertension Due to a Renin-Producing Tumor. Am J Hypertens 2018. [PMID: 29522074 DOI: 10.1093/ajh/hpx213] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2022] Open
Abstract
BACKGROUND We herein report on a 20 years old woman with stage I hypertension, who was found to carry a renin-producing tumor (RPT). METHODS Due to her young age, the patient underwent screening measurement of plasma renin and aldosterone, abdominal computed tomography (CT) angiography, and selective renal vein renin assessment to identify secondary hypertension. RESULTS The patient was screened for secondary causes of hypertension and was diagnosed with secondary aldosteronism. Therefore, she underwent an abdominal computed tomography (CT) angiography that was reported as unremarkable. Selective renal vein renin studies showed overproduction of renin in the right kidney and a re-evaluation of her CT allowed detection of an 8-mm mass in her right kidney, suggesting the presence of a RPT. Considering the technical difficulty of renal sparing surgery a CT-guided cryoablation was undertaken, which provided long-term cure of arterial hypertension and normalization of plasma active renin concentration. CONCLUSIONS RPTs usually present with a clinical phenotype featuring stage III and/or malignant hypertension and are held to be exceptionally rare. This case is unique in that it presented with stage I hypertension and a mild clinical phenotype. Moreover, to our knowledge this is the first case of RPTs shown to be safely treated with CT-guided cryoablation and found to be cured at long-term.
Collapse
Affiliation(s)
- Giuseppe Maiolino
- Clinica dell’Ipertensione Arteriosa, Department of Medicine – DIMED, University of Padua, Italy
| | - Michele Battistel
- Institute of Radiology, Department of Medicine – DIMED, University of Padua, Italy
| | - Giulio Barbiero
- Institute of Radiology, Department of Medicine – DIMED, University of Padua, Italy
| | - Valeria Bisogni
- Clinica dell’Ipertensione Arteriosa, Department of Medicine – DIMED, University of Padua, Italy
| | - Gian Paolo Rossi
- Clinica dell’Ipertensione Arteriosa, Department of Medicine – DIMED, University of Padua, Italy
| |
Collapse
|
|
15
|
Buonacera A, Stancanelli B, Malatino L. Endocrine Tumors Causing Arterial Hypertension: Pathophysiological Mechanisms and Clinical Implications. High Blood Press Cardiovasc Prev 2017; 24:217-229. [PMID: 28405904 DOI: 10.1007/s40292-017-0200-9] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/25/2017] [Accepted: 04/03/2017] [Indexed: 11/30/2022] Open
Abstract
Some tumors are a relatively rare and amendable cause of hypertension, often associated with a higher cardiovascular morbidity and mortality, as compared with that of both general population and patients with essential hypertension. This worse prognosis is not entirely related to blood pressure increase, because the release of substances from the tumor can directly influence blood pressure behavior. Diagnostic approach is challenging and needs a deep knowledge of the different neuro-hormonal and genetic mechanisms determining blood pressure increase. Surgical tumor removal can, but not always, cause blood pressure normalization, depending on how early was tumor detection, since a long-standing history of hypertension is often associated with a much weaker effect on blood pressure. Moreover, target organ damage can be affected by the substances themselves released by the tumors as well as by tumor removal. In this review we consider the phenotype and genetic features of patients with tumor-induced hypertension and focus on their diagnostic work-up.
Collapse
Affiliation(s)
- Agata Buonacera
- From the Academic Unit of Internal Medicine and Hypertension Centre, Department of Clinical and Experimental Medicine, University of Catania, c/o Cannizzaro Hospital, Via Messina 829, 95126, Catania, Italy
| | - Benedetta Stancanelli
- From the Academic Unit of Internal Medicine and Hypertension Centre, Department of Clinical and Experimental Medicine, University of Catania, c/o Cannizzaro Hospital, Via Messina 829, 95126, Catania, Italy
| | - Lorenzo Malatino
- From the Academic Unit of Internal Medicine and Hypertension Centre, Department of Clinical and Experimental Medicine, University of Catania, c/o Cannizzaro Hospital, Via Messina 829, 95126, Catania, Italy.
| |
Collapse
|