Micturition-triggered severe headache associated with bladder paraganglioma: A case report.

BACKGROUND: Paragangliomas 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. CONCLUSION: This 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.

Mastering bladder paraganglioma for optimal treatment: a case report of robot-assisted surgery.

Bladder paraganglioma is a rare neuroendocrine neoplasm, either functional or non-functional, arising from the urinary bladder. Functional variants present with catecholamine-related symptoms, while non-functional variants pose diagnostic challenges, mimicking urothelial carcinoma. Misdiagnosis risks underscore the importance of accurate identification for appropriate patient management. In this case, a 52-year-old man, diagnosed incidentally with hypertension and reported occasional post-micturition tachycardia, underwent abdominal ultrasound for known hepatic cyst follow-up, revealing an oval hypoechoic bladder mass. Initial consideration of bladder urothelial carcinoma prompted further investigation with contrast-enhanced CT scan and cystoscopy that confirmed extrinsic mass nature, and subsequent robotic-assisted partial cystectomy was performed. Histologically, the removed mass exhibited characteristic features of bladder paraganglioma. Postoperative recovery was uneventful, with resolution of post-micturition tachycardia at 1 month. Follow-up includes endocrinological evaluation and a 6-month CT scan. In conclusion, bladder paraganglioma should be considered in para-vesical mass differentials. This case highlights the importance of meticulous history collection, even in asymptomatic patients, the need for a multidisciplinary approach for accurate diagnosis and management of this rare condition, and the robotic approach as a viable option.

Case report: A novel somatic SDHB variant in a patient with bladder paraganglioma.

Background: Paragangliomas (PGL) are rare neuroendocrine tumors derived from the autonomic nervous system paraganglia. Urinary bladder paragangliomas (UBPGL) originate from the sympathetic neurons of the urinary bladder wall and represent 0.7% of all paragangliomas and <0.05% of all bladder tumors. PGL and UBPGL can be associated with SDHB, SDHD, NF1, and VHL gene variants, with the most common germline alterations found in SDHB and VHL. Case report: We report a case of a 42-year-old woman who presented with menorrhagia/hematuria, uterine leiomyomas, as well as cardiac and bladder masses. The cardiac mass was favored to be a myxoma based on clinical findings, while the bladder mass was diagnosed as UBPGL. A novel SDHB mutation (c.642G>A, p Q214Q), detected in the UBPGL, was proven to be somatic. Although this variant was seemingly synonymous, it was predicted to have a loss of function due to the splice site effect, which was further supported by the immunohistochemical loss of SDHB. Conclusion: This case highlights the challenges of diagnosing an extremely rare entity, bladder paraganglioma, with an emphasis on the multidisciplinary approach to navigate various clinical and imaging findings that may initially be misleading. In addition, a novel loss of function SDHB variant that could have been overlooked as a synonymous variant is herein reported, while also illustrating the importance of both germline and somatic mutation testing.

Bladder paraganglioma: A case of acute respiratory distress syndrome triggered by large bladder tumor transurethral resection with mild symptoms and atypical imaging.

Introduction: Bladder paraganglioma is exceedingly rare, accounting for <0.05% of bladder tumors. This is a case of paraganglioma with no symptom other than palpitations during urination, with atypical imaging, resulting in acute respiratory distress syndrome after transurethral resection of the bladder tumor. Case presentation: A 46-year-old man underwent transurethral resection of the bladder tumor for a bladder tumor 61 × 52 mm in size on contrast-enhanced computed tomography. The patient only had micturition attacks and was suspected to have urothelial carcinoma on magnetic resonance imaging. The patient had acute respiratory distress syndrome after the operation which improved conservatively. The 123Iodine metaiodobenzylguanidine scintigraphy, urinalysis, and pathological examination revealed bladder paraganglioma. Robot-assisted radical cystectomy and ileal neobladder reconstruction were performed. Conclusion: This study reported bladder paraganglioma with no symptoms other than micturition attacks in which acute respiratory distress syndrome occurred after transurethral resection of the bladder tumor.

Quantitative MRI in distinguishing bladder paraganglioma from bladder leiomyoma.

PURPOSE: To investigate the feasibility of quantitative MRI in the differentiation of bladder paraganglioma from bladder leiomyoma. METHODS: From 2014 to 2021, 11 patients with bladder paraganglioma and 14 patients with bladder leiomyoma confirmed by surgical pathology were retrospectively collected. All patients underwent multiparametric scanning with a 3.0 T MR system. Quantitative parameters including the SI-ratio on T1WI between the solid component of tumours and piriformis, ADC value and E-rate of the solid component of tumours were assessed. Independent sample t test or Mann-Whitney U test was used to compare these parameters between the two groups. The diagnostic efficiency of the parameters was examined using ROC curve analysis and the DeLong test. RESULTS: There were significant differences in SI-ratio on T1WI (P < 0.001), ADC value (P = 0.002) and the E-rate (P = 0.040) between the paraganglioma group and the leiomyoma group. The cutoff value of SI-ratio on 3 leiomyoma was 1.241, and the AUC was 1.000 (0.858-1.000). The cutoff value of the ADC value was 0.979 × 10-3mm2/s, and the AUC was 0.907 (0.717-0.987). The cutoff value of E-rate was 98.7%, and the AUC was 0.714 (0.495-0.878). The AUCs of the SI-ratio on T1WI and ADC value were significantly higher than the E-rate AUC (P = 0.015 and 0.034, respectively). CONCLUSION: Quantitative MRI can effectively distinguish bladder paraganglioma from bladder leiomyoma with the SI-ratio on T1WI or ADC value.

A case report of bladder paraganglioma and literature review.

Bladder paraganglioma is a rare tumor originated from the chromaffin cells of the bladder. We discuss a case of a 49-year-old male patient with bladder paraganglioma, including the clinical and ultrasonographic features, the histopathological and immunohistochemical manifestations, the treatment and prognosis, and the differential diagnosis of this disease. The combination of ultrasonic examination and clinical manifestations may help to make the accurate diagnosis of bladder paraganglioma, and pathological examination should be used to confirm the diagnosis.

Dynamic FDG PET/CT on bladder paraganglioma: A case report.

Paraganglioma (PGL) is characterized by equivocal clinical manifestations and arriving to a suspicion might be challenging. Nevertheless, diagnostic imaging and nuclear medicine are a fundamental part of the diagnosis and management of this particular neuroendocrine tumor (NET). We herein report a rare case of bladder paraganglioma with unusual onset and typical PET/CT characteristics that led to its recognition.

Bladder Paraganglioma: Three Cases Report and Literature Review.

BACKGROUND: 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.

Bladder paraganglioma: a case report.

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.

Imaging manifestations of bladder paraganglioma.

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.

Bladder Paraganglioma.

Teaching Point: A submucosal bladder wall lesion with high signal on T2-weighted MRI warrants blood and urine analysis to rule out a paraganglioma.

Postmicturition syndrome: a neglected syndrome dangerous for the bladder and the heart.

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.

Unusual Presentation of Bladder Paraganglioma: Comparison of (131)I MIBG SPECT/CT and (68)Ga DOTANOC PET/CT.

Extraadrenal chromaffin cell-related tumors or paragangliomas are rare, especially in the bladder, accounting for less than 1% of cases. We report a 16-year-old boy who presented with hematuria and paroxysmal headache and was found to have a prostatic growth infiltrating the urinary bladder on anatomical imaging. Iodine-131 ((131)I) metaiodobenzylguanidine (MIBG) whole-body scanning and subsequently gallium-68 ((68)Ga) DOTANOC positron emission tomography/computed tomography (PET/CT) were performed. The MIBG scan revealed a non-tracer-avid soft-tissue mass, while DOTANOC PET/CT revealed a tracer-avid primary soft-tissue mass involving the urinary bladder and prostate with metastasis to the iliac lymph nodes. He underwent surgical management; histopathology of the surgical specimen revealed a bladder paraganglioma, whereas the prostate was found to be free of tumor.

Bladder paraganglioma with renal agenesis: A possible new association and its implications in the light of REarranged in transfection gene genetics.

Pheochromocytoma/paraganglioma and renal agenesis are commonly reported conditions. Their coexistence, however, is rare, with few cases reported. We report the case of a 21-year-old male who presented with painless hematuria. He was found to have congenital absent right kidney along with bladder mass on imaging. Examination including blood pressure was normal. He underwent cystoscopy that showed a solid looking tumor on the anterior wall. Paraganglioma was suspected due to intraoperative rise in blood pressure during resection and was confirmed on histopathology. Subsequently after work up and preoperative alpha blockade, patient underwent partial cystectomy and excision of the paravesical mass. Histopathology showed paraganglioma confined to bladder wall with surgical margins free and a paravesical mass that was seminal vesicle cyst. On follow-up, patient is normotensive and asymptomatic. This coexistence of paraganglioma and renal agenesis may have a common genetic mechanism in the form of REarranged in Transfection (RET) gene mutation. This is a well-characterized gene, mutations of which are known to be associated with both conditions. Current knowledge of the role of RET gene in both conditions is reviewed to put forth RET mutation as the possible common underlying genetic mechanism along with possible clinical implications of the combination.

Episodic Hypertension With an Adrenal Mass: A Red Herring.

A 66-year-old man with symptomatic hypertensive episodes was found to have a 9-mm adrenal mass and elevated metanephrine levels. He was scheduled for an adrenalectomy for suspected pheochromocytoma. Subsequent workup revealed a large bladder mass which was found to be a paraganglioma. There is no consensus on what should be considered the standard management of primary bladder paraganglioma, including surgical technique, assessment of malignancy, and appropriate follow-up, owing to the rarity of the diagnosis.

Evaluation of sonographic features for patients with urinary bladder paraganglioma: a comparison with patients with urothelial carcinoma.

The goals of the work described here were to analyze the ultrasonographic characteristics of urinary bladder paraganglioma and to evaluate the use of ultrasonography (US) in diagnosis. Ten patients with bladder paraganglioma (3 males, 7 females) and 51 patients with urothelial carcinoma (37 males, 14 females) were enrolled. Ultrasonographic characteristics as well as clinical features were analyzed. Patients with urothelial carcinoma were significantly older than those with paraganglioma (62.9 y vs. 48.1 y, p = 0.001). The patients with paraganglioma had higher average systolic blood pressure than those with urothelial carcinoma (146.7 mm Hg vs. 130.7 mm Hg, p = 0.012). All bladder paragangliomas were solitary, and 40% were located on the dome. There was a statistically significant difference in location of lesions between the bladder paraganglioma and urothelial carcinoma groups (p = 0.014). The longest diameters in 80% of the bladder paragangliomas were in the range 1.1-3.0 cm; 60% were hypoechoic, and 40% were located in the submucosa of the bladder. Color Doppler revealed that 40% of the bladder paragangliomas were highly vascular. One patient with paraganglioma and four patients with urothelial carcinoma underwent contrast-enhanced ultrasound, which revealed rapid contrast enhancement and slow wash-out, with a non-enhanced area. Contrast-enhanced ultrasound of urothelial carcinomas revealed rapid homogeneous enhancement and variable wash-out patterns. Pre-operative ultrasonography detected all neoplasms of the urinary bladder in the 10 patients with pathologically confirmed paraganglioma, but made the correct diagnosis in only 2 cases (20%). Although there is overlap in US findings among different diseases, solitary, submucosal masses arising on the dome may be the key US imaging characteristic for bladder paraganglioma.

Bladder paraganglioma: A report of case series and critical review of current literature.

Extra-adrenal chromaffin cell-related tumours or paragangliomas are rare, especially in the bladder. In this article, we reported three different clinical cases of bladder paraganglioma, followed by a review of current literature on the pathophysiology and management of bladder paraganglioma. Case 1 involved a 23 years old female patient who complained of a 10-year history of micturition-related headaches, palpitations and diaphoresis; while in case 2, a 58 years old female patient presented with history of painless haematuria and an incidentally diagnosis of a functioning paraganglioma during endoscopic transurethral resection of bladder tumour; and lastly in case 3, a 54 years old male renal transplant recipient was referred to the urology outpatient with a suspicious bladder mass found incidentally on routine transplant workshop.