Paraganglioma of the urinary bladder-a rare, but relevant differential diagnosis

Paraganglioma of the urinary bladder is a rare tumor with characteristic histologic and immunohistochemical features. However, in our experience, it may be misdiagnosed as urothelial cancer because of 1) its frequent involvement of the muscularis propria; 2) morphology that may suggest urothelial cancer in transurethral resection specimens, particularly if there are artifactual changes induced by that procedure; 3) failure of pathologists to include it in their differential diagnosis when evaluating a bladder tumor; and 4) only a minority of the cases are associated with symptoms that might prompt consideration of the diagnosis. Distinction between paraganglioma and urothelial cancer is important because of likely different therapeutic options. In this report, we describe our experience with the histopathology of paragangliomas of the urinary bladder with emphasis on the histologic features that have led to their being misdiagnosed as conventional urothelial cancer and, most importantly, those that will help pathologists recognize this rare tumor of the bladder. Fifteen cases of paraganglioma of the urinary bladder were studied, 11 of them consult cases. They affected patients (8 male, 7 female) with a mean age of 49.5 years; only two had symptoms suggestive of the diagnosis, including hypertension during cystoscopy and episodic headache. Three consult cases were submitted with a diagnosis of "transitional cell carcinoma" and 4 with a diagnosis only of "bladder tumor." Histologically, "zellballen" and diffuse patterns were present in 12 (80%) and 3 (20%) of the cases. A delicate fibrovascular stroma was obvious in 14 (93%) cases. Other patterns included irregular nests and pseudorosette formation. Tumor necrosis, significant cautery artifact, and muscularis propria invasion were present in 1 (7%), 3 (20%) cases, and 10 (67%) cases, respectively. All 15 tumors were composed of large polygonal cells with abundant granular cytoplasm. Focal clear cells were present in 3 (20%). The nuclei were mostly uniform, although occasional pleomorphic nuclei were seen in 6 (40%) cases, and 2 (13%) had frequent pleomorphic nuclei. Mitoses were rare overall, and no abnormal mitotic figures were found. The major histologic features that led to misdiagnosis included a diffuse growth pattern, focal clear cells, necrosis, and muscularis propria invasion, with significant cautery artifact compounding the diagnostic problems. Immunohistochemically, 2 of 2 tumors were positive for neuron-specific enolase, 9 of 10 tumors for chromogranin, and 2 of 3 tumors for synaptophysin; 3 of 3 tumors were negative for cytokeratin and 1 of 1 tumor negative for HMB-45. Paraganglioma of the urinary bladder may be misdiagnosed as urothelial cancer, but a careful search for the characteristic histologic features and, if necessary, supportive immunohistochemical studies, should lead to a correct diagnosis.

An unusual case of asymptomatic non-urothelial bladder tumour

Objective To review the clinical characteristics of paraganglioma of the urinary bladder with 5 cases reprt.Methods The clinical data of 5 patients (2 males and 3 females,age 23-68 years)with paraganglioma of the urinary bladder were reported.Two cases were diagnosed by ultrasonography during health examination,1 case was found by hematuria,1 had difficulty of voiding,and 1 presented with palpitation,chest discomfort while urination.Two cases were clinical diagnosed as bladder paraganglioma,1case urachal carcinoma,and 2 cases bladder tumor.Cystoscopy showed a protruding tumor within the bladder or bladder had compressed changes.One case of tumor located in the triangle,1 in the posterior wall,1in the top,2 in the anterior wall.Three cases of biopsy were negative.Three cases of preoperative endocrine examinations showed norepinephrine significantly higher.Results Laparoscopic partial cystectomy was performed in 3 cases,open partial cystectomy in 1 case,and transurethral resection of bladder tumor (TURBT) in 1 case.One case had the complication of stress cardiomyopathy during TURBT,3 cases found intraoperative hypertensive crisis.The bleeding volume was 20-800 ml (average 126 ml),and I case received blood transfusion.During the follow-up period for 3-48 months,the blood pressure was normal,and no recurrence was found.Conclusions Bladder paraganglioma is uncommon and easily misdiagnosed.For the patients with bladder tumor,accompanied by changes in blood pressure,palpitations during urination should be highly suspicious of bladder paraganglioma.Partial cystectomy is the main treatment method. Key words: Urinary bladder neoplasms; Paraganglioma; Clinical features; Treatment

Objective To study the clinical,histopathological,immunohistochemical features and the diagnosis and treatment of paraganglioma of urinary bladder. Methods Two cases of paraganglioma of urinary bladder were treated. The first case was a male with painless haematuria. The abdominal ultrasonography and CT scan showed a 2.5 cm× 2.0 cm mass in the right anterior wall of the bladder, and urine vanillylmandelic acid elevated to 17. 9- 31. 3 μmol/24 h (normal range 10- 35 μmol/24 h). The second case was a female who presented with 8 years history of headache and palpitation after voiding. Abdominal ultrasonography and CT scan showed a 2. 6 cm× 1.5 cm mass in the left wall of the bladder, and her urine vanillylmandelic acid was 35.3-43.3 μmol/24 h. Results One patient underwent transurethral resection (TURBT) and the other underwent partial cystectomy.The two cases were diagnosed as bladder paraganglioma by pathological examination. Immunohistochemically, the tumor cells were positive for CgA, Syn, NSE and S-100. No evidence of recurrence was detected during follow-up at 3 months and 3 years. Conclusions Paraganglioma of urinary bladder should be considered as a low grade malignancy. Partial cystectomy should be recommended. The diagnosis depends on clinical symptoms, pathological and immunohistochemical results. Key words: Urinary bladder neoplasms; Paraganglioma; Pathology, clinical

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.

BackgroundNonfunctional 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.ConclusionsNonfunctional 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.

Objective: We report two incidental cases of bladder paraganglioma and a review of the literature published to date. Methods: Medline was searched for the last 10 years for the terms "paraganglioma" and "urinary" and "bladder". Results: Bladder paraganglioma (BP) is extremely rare. It represents 10% of all paragangliomas and concerns less than 0.06% of bladder tumors. Often discovered by chance, it can nevertheless be symptomatic, particularly during micturition. The most common symptoms are hematuria, hypertension and headache. About 14% of these tumors are malignant and radio-chemically resistant. Surgery is therefore the cornerstone of treatment. We recommend, cystoscopy, partial cystectomy and regular follow-up based on regular cystoscopy and imaging. Their rarity explains why, at present, there are no recommendations for treatment and monitoring. However, their risk of malignancy recurrence and metastasis requires regular and long-term follow-up. Conclusions: Bladder paragangliomas are rare tumors. Treatment should be based on partial cystectomy and their follow-up should be regular.

Introduction: Paraganglioma of the urinary bladder (PUB) is an extremely rare extra-adrenal catecholamine-secreting neuroendocrine tumor, accounting for only 0.05% of all bladder tumors and 1% of all pheochromocytomas. The clinical presentation of PUB can be diverse and challenging to diagnose. Case Presentation: This case report presents a 37-year-old man with post-micturition palpitation, headache, and sweating, with no history of hematuria or other irritative urinary symptoms. Ultrasound and contrast-enhanced computed tomography revealed a suspicious mass in the right posterolateral wall of the urinary bladder. Despite normal functional hormonal tests, the diagnosis of PUB was confirmed after surgical enucleation and histopathological examination. Conclusion: This report emphasizes the importance of considering PUB as a differential diagnosis in patients with post-micturition symptoms and paroxysmal hypertension, as well as the need for a multidisciplinary approach in the evaluation and management of such rare and complex cases. Early recognition and surgical intervention are crucial for optimal management and favorable clinical outcomes.

BackgroundParaganglioma of the urinary bladder (Pub) is rare and presents with clinical symptoms caused by catecholamine production and release. The typical symptoms of Pub are hypertension, macroscopic hematuria, and a hypertensive crisis during micturition. The average size of detected Pubs is approximately 3 cm. Herein, we report a case of a large Pub in which the symptoms were masked by oral medication, precise preoperative diagnosis was difficult, and intraoperative confirmation of tumoral adhesion to the rectum resulted in hypertensive attacks during surgery.Case presentationA 64-year-old Japanese male with a history of hypertension and arrhythmia controlled with oral medication presented with a large tumor in the pelvic region, detected on examination for weight loss, with no clinical symptoms. Computed tomography and magnetic resonance imaging revealed a tumor measuring 77 mm in diameter in the posterior wall of the urinary bladder. The border with the rectum was unclear, and the tumor showed heterogeneous enhancement in the solid part with an enhancing hypodense lesion. Cystoscopy revealed compression of the bladder trigone by external masses; however, no tumor was visible in the lumen. Endoscopic ultrasonography-guided fine-needle aspiration revealed CD34-positive spindle-shaped cells in the fibrous tissue, suggestive of a mesenchymal neoplasm. The tumor was suspected to be a gastrointestinal stromal tumor, and surgery was performed. After laparotomy, we suspected that the tumor had invaded the rectum, and total cystectomy and anterior resection of the rectum were performed. Histologically, the tumor cells had granular or clear amphophilic cytoplasm with an oval nucleus and nests of cells delimited by connective tissue and vascular septations. Immunohistochemically, the tumor was positive for chromogranin A, CD56, and synaptophysin, and a diagnosis of paraganglioma of the urinary bladder was confirmed. There was no tumor recurrence at the 7-month follow-up.ConclusionThis case highlights the importance of careful examination of pelvic tumors, including endocrine testing, for detecting paraganglioma of the urinary bladder in patients with a history of hypertension or arrhythmia.

BackgroundMetastatic pheochromocytomas and paragangliomas are rare neuroendocrine tumors with a poor prognosis. Bladder paraganglioma concomitant with urothelial papilloma is even rarer. However, the rate of tumor response to cyclophosphamide–vincristine–dacarbazine (CVD) chemotherapy and 5-year overall survival for patients with metastatic PPGLs remained lower. We described, for the first time, a case of a patient with multiple metastatic bladder PGL who received octreotide LAR combined with CVD chemotherapy after urological surgery and then octreotide therapy was continued during follow-up.Case presentationA 43-year-old male patient was admitted to the urology department for frequent micturition syncope concomitant with malignant hypertension. Preoperative findings were elevated levels of normetanephrine in 24-h urine or plasma. CT and MRI indicated diagnosis of suspicious bladder paraganglioma. Transurethral resection of bladder tumor combined with laparoscopic partial cystectomy was performed successfully after preoperative phenoxybenzamine with aggressive volume repletion for 7 days. The result of postoperative pathology was immediate-risk functional bladder paraganglioma (T2N0M0, Stage II) concomitant with urothelial papilloma, and the immunohistochemistry results of PPGL were positive for Ki-67 (15%), SDHB, CgA, and SSTR2. The patient achieved enhanced recovery with normal urination and no syncope after surgery. However, the results of 18F-FDG and 18F-DOTATATE PET/CT found that the metastatic localizations of bladder PGLs were in the liver, lung, and bones at the 8th month after surgery. The patient received octreotide long-acting repeatable plus six courses of CVD chemotherapy for 6 months, and then octreotide therapy was continued every 3 months until now. Metastatic localizations were stable in CT scans, and vanillylmandelic acid in 24-h urine was maintained at lower levels during follow-up.ConclusionOctreotide long-acting repeatable plus CVD chemotherapy after surgery could achieve stable disease in the case with multiple metastatic bladder PGLs, and the following octreotide therapy could maintain a state of stable disease during the period of 6-month follow-up.

Paraganglioma of the urinary bladder is rare, accounting for <0.05% of all bladder tumors. Common clinical findings in patients with bladder paraganglioma include hematuria and intermittent hypertension during urination, along with generalized symptoms due to increased levels of catecholamines. Although unsuspected bladder paraganglioma may result in intraoperative hypertensive crises, these may be avoided if characteristic imaging signs are observed. The present study reports a case in which a patient with unsuspected paraganglioma experienced a severe hypertensive episode during cystoscopic tumor resection. Although this case had typical computed tomographic characteristics of the bladder paraganglioma, the possibility of the paraganglioma pre-operatively was not taken into account.

Partial Cystectomy of Paraganglioma of the Urinary Bladder Before Living Kidney Transplantation: Case Report

Paraganglioma of the urinary bladder is rare, comprising less than 0.06% of all vesical tumors. The most common symptoms include hypertension, hematuria, palpitation, and postmicturition syncope. A 72-year-old man without a history of hypertension presented with painless gross hematuria and acute urine retention. Computed tomography revealed a solid lesion measuring 2cm×2cm×2cm with muscular invasion of the right lateral wall of the urinary bladder. Transurethral resection was performed. Immunohistochemical studies revealed positive staining for S-100 and chromogranin A, findings consistent with the features of paraganglioma. At 6- month follow-up, cystoscopy revealed no disease recurrence.

Bladder paraganglioma: Case report and review of the literature

Bladder paragangliomas (bPGLs) are extremely rare tumors, and we present a unique case of metastatic bPGL that developed within three months. Frequently, these tumors are related to a hereditary syndrome due to germline mutations in succinate dehydrogenase B (SDHB). A unique challenge in this case was related to social determinants of health, which, in this patient, were associated with difficulty maintaining appointments. In treating a patient with hematuria, it is important to consider paraganglioma (PGL) as a differential diagnosis to optimize treatment.