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BackgroundParagangliomas are rare neuroendocrine tumors, especially in the bladder. Although many paragangliomas are non-functional, functioning paragangliomas present with many non-specific signs and symptoms, such as hypertension and headache, complicating their diagnosis. Here, we report a case of micturition-induced severe headache associated with a bladder paraganglioma. Case description: This report describes a severe headache disorder triggered by micturition and associated with a bladder paraganglioma in a middle-aged woman. Her pain occurred consistently after micturition, beginning from the left side of the nose and gradually extended to the forehead and the parietal and occipital regions. The headaches lasted 5–10 min. Removal of the paraganglioma completely eliminated the patient’s pain syndrome.ConclusionThis case elucidates the association between micturition-triggered headaches and bladder paragangliomas. The presence of a post-micturition severe headache should suggest the possibility of a bladder paraganglioma.

Background Nonfunctional bladder paragangliomas is a rare urological disease. It may present clinical, radiology and pathological features similar to bladder cancer, Only scarce reports have been reported. Urologist must identify this generally benign neuroendocrine neoplasm to avoid misdiagnosis. Case presentation A 62-year-old female presented the outpatient department of our hospital with the symptoms of stomachache, frequent micturition, and urination pain for 20 days. Diagnosed with high blood pressure 1 year ago, administered Amlodipine besylate tablets 5 mg po qd occasionally, did not check blood pressure; denied any tumor observation in the family history. Color ultrasound of the urinary system showed a 38 mm × 34 mm hypoechoic mass on the right side of the bladder, CDFI: in the masses, blood supply was sufficient. Cystoscope showed bladder occupying lesion. Biopsy diagnosis: papillary polypoid cystitis was suspected as a malignant change (Fig. 3a). Then, the patient was admitted to our urological department. Further, computer tomography urography considered bladder cancer. Cystoscopy and biopsy failed to define the nature of the lesions in our outpatient department, which prompted a transurethral resection of the bladder tumor. histopathological and immunohistochemical results were diagnosed as bladder paragangliomas. For the reason, the tumor was removed by partial resection of the bladder. The postoperative recovery and follow-up were uneventful. Conclusions Nonfunctional bladder paragangliomas are occasionally found on imaging studies with the symptoms of urinary tract infection or/and intermittent painless hematuria. It may present clinical, radiology and pathological features similar to bladder cancer, so knowledge of this generally benign neuroendocrine neoplasm is of great importance to avoid misdiagnosis. It should be accompanied by the clinical and pathological characteristics of the patient and image changes. Partial resection of the bladder can effectively treat this disease.

Background: Pheochromocytoma and paragangliomas (PPGL) are known as tumors with the highest level of heritability, approximately 30% of all cases. Clinical practice guidelines of PPGL recommend genetic testing for germline variants in all patients. In this study, we used whole exome sequencing to identify novel causative variants associated with PPGL to improve the detection of rare genetic variants in our cohort.Methods: Thirty-six tested negative for pathogenic variants in previous Sanger sequencing or targeted gene panel testing for PPGL underwent whole exome sequencing. Whole exome sequencing was performed using DNA samples enriched using TruSeq Custom Enrichment Kit and sequenced with MiSeq (Illumina Inc.). Sequencing alignment and variant calling were performed using SAMtools.Results: Among previously mutation undetected 36 patients, two likely pathogenic variants and 13 variants of uncertain significance (VUS) were detected in 32 pheochromocytoma-related genes. SDHA c.778G>A (p.Gly260Arg) was detected in a patient with head and neck paraganglioma, and KIF1B c.2787-2A>C in a patient with a bladder paraganglioma. Additionally, a likely pathogenic variant in BRCA2, VUS in TP53, and VUS in NFU1 were detected.Conclusion: Exome sequencing further identified genetic alterations by 5.6% in previously mutation undetected patients in PPGL. Implementation of targeted gene sequencing consisted of extended genes of PPGL in routine clinical screening can support the level of comprehensive patient assessment.

Background To investigate the imaging findings of bladder paraganglioma (BPG) and improve the imaging recognition and diagnostic accuracy of the disease. Methods The clinical and imaging data of 10 cases of BPG confirmed by operation and pathology were retrospectively analyzed from the aspects of tumor location, size and morphology, presence or absence of necrosis, cystic change and calcification, and enhancement methods. Results Of the 10 cases, 7 cases underwent basal and contrast enhanced computed tomography (CT) scan examination, 1 case underwent basal and contrast enhanced MRI scan examination, and 9 cases underwent ultrasonography examination; 5 cases were located in the right wall of bladder, 3 cases were located in the left wall, and 2 cases were located in the bottom wall; 2 cases showed necrosis, 1 case had cystic degeneration, and 2 cases had arcuate and semicircular calcification; both contrast enhanced CT and MRI examinations showed significant post-contrast enhancement; in 9 cases of ultrasonography examination, 5 cases had hypoechoic mass, 3 cases had moderate echoic mass, and 1 case had hyperechoic mass. Conclusions The imaging findings of BPG have certain characteristics, and combining with clinical history is helpful for accurate preoperative diagnosis.

Yaoji Yuan,1,2,* Zhengming Su,1,2,* Rui Zhu,1,2 Xiezhao Li,1,2 Guibin Xu1,2 1Department of Urology, The Fifth Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong, People’s Republic of China; 2Key Laboratory of Enhanced Recovery After Abdominal Surgery, Guangzhou, Guangdong, People’s Republic of China*These authors contributed equally to this workCorrespondence: Guibin XuDepartment of Urology, The Fifth Affiliated Hospital of Guangzhou Medical University, 621 Gangwan Road, Guangzhou, Guangdong, People’s Republic of ChinaEmail gyxgb@163.comBackground: Bladder paraganglioma (BPG) is one of the rare neuroendocrine neoplasms that develops from neural crest cells. It categorizes into functional and non-functional types based on the catecholamines secretion. Currently, functional BPG is predicted in advance based on signs and symptoms of catecholamine excess, such as hypertension and “micturition attacks”. However, it is often overlooked because of its rareness. Misdiagnosis of a functional tumor may increase the risk of surgical intervention.Case Presentation: We reported 3 cases of BPG that they were admitted to the hospital due to abdominal pain or gross hematuria. Computed tomography (CT) scans showed space-occupying lesions in the bladders with diameters less than 3cm. There were no typical catecholamine excess symptoms before surgical intervention. Postoperative pathology confirmed BPG after removal of the tumor. We also analyze 69 cases of BPG that has been reported and found that 78.0% cases were functional among the tumors larger than 3cm.Conclusion: Bladder tumors larger than 3cm in diameter can serve as an additional predictor of functional BPG. Patients who are suspected should undergo magnetic resonance imaging (MRI) scans, 123/131 metaiodobenzylguanidine (MIBG) scan, and have their catecholamine levels tested. Once the diagnosis is confirmed, patients should be started on fluid replacement therapy and adrenergic blockade to abate the disorders associated with catecholamine excess.Keywords: bladder paraganglioma, cases report

Bladder paragangliomas (PGLs) are extremely rare catecholamine-producing neuroendocrine tumors. They arise more frequently in the trigone and have unpredictable depth and behavior. Most cases typically present with a well-defined set of symptoms triggered by micturition or bladder overdistension. Besides long-term follow-up, they are usually managed by either transurethral resection (TUR) or partial cystectomy (PC). However, about 25% of all documented cases do not manifest clinically, raising both diagnosis and management challenges. This report describes an unusual case of a misdiagnosed, functional PGL arising on the bladder dome, which was fortuitously detected in a 21-year-old female during a fertility workup. Owing to its hypervascular nature and submucosal location, bladder PGL was suspected on ultrasound and CT findings and successfully diagnosed before surgery through biochemical confirmation. It was managed by cystoscopy-guided laparoscopic partial cystectomy (LPC) with good oncological and urinary outcomes, as well as no compromise of her fertility potential. To our best knowledge, this is the first case to be incidentally detected on transvaginal ultrasound during evaluation for infertility. This case also stresses the importance of considering PGL in the differential diagnosis of atypical bladder tumors, as well as conservative management through simultaneous laparoscopy and cystoscopy, when approaching young patients with large functional PGL.

Introduction:Bladder paraganglioma is a rare tumour of chromaffin cell origin. Few cases have been reported in the literature.Case report:A 59-year-old female with no known co-morbidities presented with catecholamine-related symptoms, haematuria, elevated blood pressures and raised urine metanephrines. A sonar and computed tomography scan confirmed a bladder mass shown to be active on iodine-131 meta-iodobenzylguanidine. Partial cystectomy was performed.Conclusion:Although bladder paraganglioma is rare, clinicians need to be aware of how such cases may present and the necessary work-up to confirm the diagnosis. Surgery is the treatment of choice, and long-term follow-up of these patients is necessary to detect recurrence or metastases.Level of evidence:4.

A 66-year-old woman was referred to our hospital because a bladder tumor was detected by abdominal ultrasonography. Although she was asymptomatic, cystoscopy showed the nodular sessile tumor in the bladder. We performed transurethral resection of the bladder tumor (TURBT) and histological examination revealed paraganglioma of the urinary bladder. Computed tomography (CT) and metaiodobenzylguanidine (MIBG) scintigraphy did not reveal any other lesions of paraganglioma. One month later, we performed a second TURBT, but the histological examination revealed no residual tumor. She has been followed up for 3 years after operation without any evidence of recurrence. Paraganglioma of the urinary bladder originates from chromaffin tissue of the sympathetic nervous system associated with the urinary bladder wall. For bladder submucosal tumor, we should consider bladder paraganglioma. And second TURBT is a useful option for the evaluation of the residual tumor.

Paragangliomas are neuroendocrine tumours of extra-adrenal origin. Primary bladder paragangliomas are rare and have variable functional status, which presents a challenge in their management by minimally invasive surgery. Surgical resection necessitates minimal intraoperative handling of tumour, minimising physiological deterioration. Here in, a case is reported of incidentally detected locally advanced bladder paraganglioma which was managed successfully with robot-assisted radical cystectomy and intracorporeal ileal neobladder formation.

A 68-year-old woman presented with episodes of headache, palpitations, sweating and poorly controlled hypertension for the past 6 years. These symptoms were, at times, associated with micturition, and there were few episodes of micturition syncope as well. She had elevated 24-hour urinary normetanephrine and was found to have a paraganglioma arising from the urinary bladder infiltrating the sigmoid colon. She underwent laparotomy with excision of the bladder paraganglioma, following which her symptoms subsided. Paragangliomas are extra-adrenal catecholamine-producing tumours. Bladder paragangliomas need to be considered when evaluating hypertensive patients with headache, palpitations or syncope related to micturition.

Bladder paragangliomas are very rare tumors that are often misdiagnosed. The present study retrospectively analyzed the clinical data and follow-up results of patients with bladder paraganglioma that were treated surgically in Peking University Shenzhen Hospital between 2012 and 2018, and reviewed the relevant literature. A total of 4 patients with bladder paraganglioma were treated surgically from 2012 to 2018. All patients were female with ages ranging from 28 to 54 years old (average, 47.25). A total of one patient exhibited micturition attacks and palpitations, and one other exhibited gross hematuria. A total of two patients exhibited while no obvious symptoms. One patient exhibited elevated plasma catecholamine, was well prepared with medicine and underwent partial cystectomy, and 3 patients were not successfully diagnosed with paraganglioma before surgery. These patients received transurethral resection and 2 had distinct blood pressure elevation during operation. After surgery, the patients were followed-up for 5-69 months, during which all had normal blood pressure and none experienced recurrence. Bladder paragangliomas exhibit a variety of clinical presentations, which can be nonspecific for the non-functional ones, therefore may easily lead to misdiagnosis. Surgeons must fully consider the possibility of this disease when dealing with non-epithelial bladder tumors. Surgical resection is an effective treatment. For cases successfully diagnosed before surgery, partial cystectomy should be a treatment priority.

Tumors of chromaffin cells derived from the embryonic neural crest are classified as pheochromocytomas when located in the medulla of the adrenal glands and paragangliomas when located externally to it. Bladder paragangliomas constitute less than 1% of all catecholamine-secreting neuroendocrine tumors. Usually, these tumors are functional and symptomatic, but in other cases, they can be silent. We present an interesting case of a patient with post-micturition catecholaminergic crises that went undiagnosed for several years until found to have a bladder paraganglioma. A 60-year-old male with a past medical history of TIA, HTN, CAD, and ischemic cardiomyopathy presented with post-micturition headaches since age 14. Despite work up, no etiology was found. Eventually, the patient was referred to mental health for what was thought to be somatic symptoms. In 2010, he was diagnosed with hypertension and started keeping daily blood pressure (BP) logs, which showed BP fluctuation as high as 260s/110s with high-volume micturition, yet normotensive at other times. This led to imaging and cystoscopy which revealed a 6-cm bladder lesion. Surgical resection was completed, and pathology showed paraganglioma with positive margins and lymphovascular invasion. He was lost to follow up until 2017 when he presented with hypertensive crisis. CT and octreotide scan showed numerous osseous lytic lesions concerning for metastases. Laboratory testing confirmed excess catecholamines. He was referred to oncology, and after consideration of several treatment options, the patient was started on Xgeva and Sandostatin LAR. However, he passed away shortly thereafter. Bladder paragangliomas are very rare and account for 6% of extra-adrenal pheochromocytomas. They occur more frequently in women than in men and clinically present mainly during the third decade of life. The patient typically suffers from hypertensive crises that may be accompanied by headache, palpitations, hot flushes, and sweating. These crises are mainly provoked by micturition, overdistention of the bladder, defecation, sexual activity, ejaculation, or bladder instrumentation. If there is high suspicion, biochemical and functional imaging workup should be performed. Surgery is the mainstay of the treatment and requires total excision. If diagnosed preoperatively, a partial cystectomy is preferred over trans-urethral resection as the majority of these tumors extend in the deep layers of the detrusor muscle. Because they are likely to recur and to metastasize, annual follow up with a measurement of plasma and urinary catecholamine levels and cystoscopy are essential. A functional imaging study (Ga-DOTATE scan) should be done to locate recurrence if symptoms reappear or catecholamine resurgence occurs.

Bladder Paraganglioma is a rare type of bladder tumor (0.06%). It is typically benign and the most common symptoms are hematuria, hypertension and headache. About 14% of these tumors are malign and consequently radio and chemoresistants. Therefore, surgery is the mainstay of treatment. As they are likely to recur and to metastize lifelong follow-up is required. The authors report a rare case of a 53 years old man with hematuria and a previous history of micturition syncope who was diagnosed with bladder lesion. During the transurethral ressection of bladder he became severely hypertensive. Plasma metanephrines, and urinary vanillylmandelic acid, were still high and the exams suggested residual tumor. The patient underwent radical cistoprostatectomy. After 4 years of follow-up the patient remains disease free.

Extra-adrenal pheochromocytoma is called paraganglioma. Paraganglioma near the ureterovesical junction can be confused with urothelial carcinoma in a ureterocele. Urinary metanephrine can be an indicator for bladder paraganglioma. Metaiodobenzylguanidine scintigraphy is an excellent method not only for distinguishing bladder paraganglioma from other submucosal mesenchymal tumors but also for detecting multifocal lesions. In the present case, we did not perform a preoperative metaiodobenzylguanidine scan because the patient was asymptomatic and urinary metanephrine was negative. Partial cystectomy with ureteroneocystostomy was performed for curative treatment because the tumor was very close to the ureteral orifice.

Postmicturition syndrome refers to symptoms caused by overdistension of the bladder or micturition. Bladder paraganglioma is a rare neuroendocrine neoplasm, which arises from the chromaffin tissue of the sympathetic nervous system embedded in the muscle layer of the bladder wall. Clinical presentation of catecholamine-secreting paragangliomas may mimic that of hyperfunctioning adrenal pheochromocytoma. Typical symptoms such as sweating, palpitations, headache, nausea, hypertension, or flushing are due to catecholamine release and are related to micturition or bladder overdistension. We herein report the case of a 22-year-old woman admitted to the Emergency Department because of cranial trauma secondary to a car accident. She referred history of micturition-related headache, nausea, sweating, and increase in blood pressure since she was 13 years old. The neurological investigation was normal. No urogenital tract investigation was performed and, on admission, blood pressure was 190/125 mmHg. During hospitalization, abdominal ultrasonography, performed to rule out secondary hypertension, unexpectedly showed a large vascular soft tissue mass in the bladder wall, compatible with a paraganglioma. Twenty-four hours of urinalysis of catecholamines revealed high values of urine metanephrines. Abdominal magnetic resonance imaging and histopathological evaluation of the surgical specimen, following resection of the bladder lesion, confirmed the diagnosis. Our case underlines the importance not to underestimate symptoms compatible with postmicturition syndrome, especially in young patients, to make early diagnosis of bladder paraganglioma.

Hypertensive heart disease with biventricular cardiac failure is not common in clinical practice. This diagnosis requires an extensive diagnostic search. We present the clinical case of the male patient of 38 aged. He was admitted to the clinic with heart failure 3-4 NYHA class. EchoCG revealed symmetric hypertrophy of the left ventricle up to 18 mm without its dilatation, a decrease in ejection fraction up to 42%, restrictive hemodynamics, overload of the right chambers, severe pulmonary hypertension (60 mm Hg). The clinical status included persistent arterial hypertension (180-220 and 120-150 mm Hg), effusion in both pleural cavities and pericardium, ascites, renal failure. During examination (multispiral computed tomography, magnetic resonance imaging, scintigraphy with 131I-MIBG), bladder paraganglioma was diagnosed (normatenafrin 1468 μg/day). The resection of the tumor was performed, according to immunohistochemical research – neuroendocrine carcinoma, G1. After 3 months a partial regression of hypertension and cardiac failure was observed with the preservation of a high level of creatinine. The criteria and differential diagnosis of the hypertensive heart disease and the syndrome of primary myocardial hypertrophy, diagnostics of the urinary bladder paraganglioma, complex mechanisms of myocardial damage within the pheochromocytoma and its prognosis are discussed.

Background Paraganglioma (extra-adrenal pheochromocytoma) of the bladder is a very rare disease, accounting for 0.06% of all bladder tumors. Optimal management of bladder paraganglioma before kidney transplantation is unknown. We report a case of partial cystectomy for urinary bladder paraganglioma before living kidney transplantation. Case Presentation A 59-year-old man with a 27-year history of hemodialysis was referred to our department for further examination of a bladder tumor detected during pre-transplantation testing. Cystoscopy revealed a submucosal tumor on the right side of the bladder. The patient experienced a hypertensive crisis during transurethral resection of the bladder tumor. Endocrinologic and pathologic examinations confirmed the diagnosis of paraganglioma in the urinary bladder. A partial cystectomy was performed before kidney transplantation. Nine months after partial cystectomy, the patient underwent AB0-incompatible living kidney transplantation from his spouse. No disease recurrence or graft rejection was observed 12 months after the transplantation. Conclusions To our knowledge, this is the 1st report on the management of paraganglioma in the urinary bladder before living kidney transplantation. Kidney transplantation after partial cystectomy is an option that may be considered in patients with paraganglioma of the urinary bladder, with careful observations of bladder function and vesicoureteral reflux to the grafts.

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Pheochromocytoma is a neoplasm, which develops from cells of the chromaffin tissues that are derived from the ectodermic neural system and mostly situated within the adrenal medulla. Approximately 15% of pheochromocytoma cases arise from extra-adrenal chromaffin tissue. Pheochromocytoma of the bladder is rare and accounts for less than 0.06% of all bladder neoplasms and less than 1% of all pheochromocytomas. We report a case of a young woman who presented with uncontrolled hypertension, recurrent urinary tract infections and micturition attacks and was found to have a metastatic bladder paraganglioma. In addition, we provide a summary table of the clinical manifestations of paragangliomas based on anatomic locations.

Background: Paragangliomas (PGLs) are catecholamine secreting neuroendocrine tumors originating from extra-adrenal neural crest cells. Urinary bladder paragangliomas (UBPGLs) are extremely rare, accounting for only 0.06% of all bladder tumors. Sporadic UBPGLs associated with Succinate Dehydrogenase B (SDHB) mutation have a higher malignant potential versus patients with a positive family history due to a lack of screening. We present a case of sporadic UBPGL detected early due to follow-up of an incidental bladder mass. Clinical Case: A 31 year-old female presented to the ER with presyncope and lower abdominal pain while running. On arrival she was mildly hypertensive with BP 140/88 mmHg, HR was 98 beats/min, other vital signs were stable. Denied headaches, diaphoresis, or weight loss. No known personal or family history of any endocrine disorders or cancer. CT abdomen pelvis performed for the evaluation of her abdominal pain revealed a large fibroid along with an unexpected finding of a 3 cm enhancing lesion in the right bladder wall, suggestive of a PGL. 24 hr urine total catecholamines and plasma total metanephrines were elevated at 284 mcg (normal range 26-121 mcg/24h), and 386 pg/mL (normal Conclusion: This case illustrates the importance of following up on incidental radiographic findings and adds to the limited literature on sporadic UBPGLs, an uncommon, yet potentially life-threatening tumor. The diagnosis of these tumors is especially challenging when patients present with non-specific symptoms. Fortunately, in our case, the imaging study clued us into the possibility of a PGL. SDHB mutation associated PGLs, in particular, are notorious for their late presentation with metastasis and associated tumors. Hence, timely detection with close follow-up of these patients will likely have an impact on their mortality.

A 66-year-old woman was referred to our hospital because a bladder tumor was detected by abdominal ultrasonography. Although she was asymptomatic, cystoscopy showed the nodular sessile tumor in the bladder. We performed transurethral resection of the bladder tumor (TURBT) and histological examination revealed paraganglioma of the urinary bladder. Computed tomography (CT) and metaiodobenzylguanidine (MIBG) scintigraphy did not reveal any other lesions of paraganglioma. One month later, we performed a second TURBT, but the histological examination revealed no residual tumor. She has been followed up for 3 years after operation without any evidence of recurrence. Paraganglioma of the urinary bladder originates from chromaffin tissue of the sympathetic nervous system associated with the urinary bladder wall. For bladder submucosal tumor, we should consider bladder paraganglioma. And second TURBT is a useful option for the evaluation of the residual tumor.

Rationale: Bladder paraganglioma is characterized by headache, palpitations, hypertension, blurred vision, or sweating during voiding. Transurethral holmium laser resection is a safe and efficacious alternative method for the resection of bladder neoplasms. Patient concerns: A 24-year-old female had a 2-year history of intermittent headaches, palpitation, and sweating during micturition. Diagnosis: Physical examination revealed a rise in the patient's blood pressure to 180/90 mmHg after micturition. Laboratory examination found that the blood catecholamine metabolites were significantly increased. Abdominal ultrasound and computed tomography (CT) scan indicated a 37 mm × 31 mm paraganglioma situated at the right anterolateral wall of the bladder. A diagnosis of bladder paraganglioma was considered based on a comprehensive evaluation of the physical examination, laboratory examination, ultrasound and computerized tomography scan. Interventions: Preoperative oral administration of a nonselective α-adrenergic receptor antagonist (phenoxybenzamine, 10 mg three times a day,) accompanied by a high-sodium diet and generous fluid intake, was initiated 2 weeks before the surgery to stabilize intraoperative hemodynamics. As the patient was newly married and nulligravid, management with transurethral resection was considered superior to open or partial cystectomy and was selected as the treatment method. Outcomes: Transurethral holmium resection of the bladder paraganglioma was successfully performed with blood loss less than 20 ml and well-controlled intraoperative blood pressure. The 1-year follow-up results demonstrated well-controlled symptoms. Cystoscopy and evaluation of blood catecholamine metabolites revealed no disease recurrence. Lessons: Transurethral holmium laser resection is a good alternative approach for the resection of bladder paraganglioma, given its advantages of safety and efficacy.