Paraganglioma Of Urinary Bladder Research Articles

Urinary Bladder and Left Hypogastric Artery Paraganglioma. A Case Report and Literature Review

Urinary Bladder and Left Hypogastric Artery Paraganglioma. A Case Report and Literature Review

A Rare Case Of Functional Extra-Adrenal Urinary Bladder Paraganglioma In A 19-Year-Old Female Managed With Partial Cystectomy And Ureteral Reimplantion Using A Multidisciplinary Approach

ABSTRACT Objective: We report the multidisciplinary management of an unusual case of functional urinary bladder paraganglioma in a 19-year-old hypertensive female patient. Methods: We present the complete clinical presentation, imaging, operative details, histopathology, and follow-up of an unusual case of functional urinary bladder paraganglioma. Results: This case was unique because it presented as a functional tumor arising from urinary bladder in a young patient with uncontrolled hypertension. After biochemical, anatomical, and functional imaging, the diagnosis of a functional urinary bladder paraganglioma was confirmed. Adequate preoperative medical management was given to stabilize her blood pressure. Surgery was performed after proper optimization and explaining all of the high risk factors and expected complications of the procedure. Meticulous surgical planning was done to achieve complete tumor resection without any major complications or compromising the patient's fertility. Genetic analyses of...

Urinary Bladder Paragangliomas: Analysis of Succinate Dehydrogenase and Outcome

Paragangliomas of the urinary bladder can arise sporadically or as a part of hereditary syndromes including those with underlying mutations in the succinate dehydrogenase (SDH) genes, which serve as tumor suppressors. SDH deficiency can be screened for by absence of immunohistochemical detection of SDHB. In this study of 11 cases, clinical follow-up was available for 9/11 cases. The cases were reviewed and graded based on the grading system for adrenal pheochromocytomas and paragangliomas (GAPP) criteria. Immunohistochemistry was performed for Ki67 and SDHB. Proliferative index was calculated by quantification of Ki67-positive cells at hot spots. The medical record was accessed for documentation of germline SDH mutations. Urinary bladder paragangliomas had a female predilection (8/11 cases), and 5/11 cases exhibited metastatic behavior. Patients with metastatic disease tended to be younger (mean age 43 vs 49years), have larger lesions (5.8 vs 1.5cm), and presented with catecholamine excess (4/4 vs 2/6 patients with non-metastatic lesions). Patients with metastatic disease had a higher mean Ki67 proliferation rate (4.9 vs 1.3%) and GAPP score (mean of 5.8 vs 3.8) (p = 0.01). IHC for SDHB expression revealed loss of expression in 2/6 cases of non-metastatic paragangliomas compared to 4/5 patients with metastatic paragangliomas. Interestingly, of these four patients, two had a documented mutation of SDHB, one patient had a SDHC mutation, and another patient had a history of familial disease without mutation analysis being performed. Our study, suggests that SDH loss was suggestive of metastatic behavior in addition to younger age at diagnosis, larger tumor size, and higher Ki67 proliferation rate and catecholamine type.

Atypical Presentation of Vesical Paraganglioma

Paragangliomas are extra adrenal pheochromocytoma arising from the chromaffin tissue of sympathetic nervous system. It accounts for 15% of all the pheochromocytomas. Paraganglioma of urinary bladder is an extremely rare entity and difficult to diagnose in absence of typical symptoms of catecholamines excess. They usually have typical symptoms of hypertensive episodes specifically during voiding or sexual activity. Calcification within paraganglioma is even rare entity and often mimics vesical calculus. Partial cystectomy remains the treatment of choice and confirmation of diagnosis requires immunohistochemical staining. Here we report a case of calcified paraganglioma of urinary bladder with atypical presentation and review of pertinent literature.

Paraganglioma of the urinary bladder with chromosome duplications detected by fluorescence in situ hybridization in urine exfoliated cells: A case report.

Paragangliomas of the urinary bladder are rare neoplasms derived from chromaffin tissue with a chromosomal imbalance. Their preoperative diagnosis and assessment of malignant potential remain significant challenges for urologists. The current report presents the case of a 34-year-old male who presented with a history of paroxysmal gross hematuria lasting for 7 months. Chromosome duplications were detected by fluorescence in situ hybridization (FISH) in urine exfoliated cells, and the diagnosis was confirmed by histopathology following a transurethral resection. To the best of our knowledge, this is the first reported case of urinary bladder paraganglioma in which chromosomal duplications were detected by FISH in urine exfoliated cells. This may be helpful to its differential diagnosis and malignant potential determination.

Non-functioning paraganglioma occurring in the urinary bladder: A case report and review of the literature.

Paraganglioma, also termed extra-adrenal pheochromocytoma, may be observed at the base of the skull and neck as well as within the mediastinum and periaortic region. The clinical symptoms of paraganglioma of the urinary bladder include intermittent hypertensive attacks, micturition, headaches and palpitations due to high catecholamine levels; these types of paragangliomas are extremely rare. However, certain bladder paragangliomas do not present with any of these symptoms; thus, surgeons are not pre-warned on how to prepare for their resection. Surgery to remove pheochromocytomas may therefore result in an intraoperative hypertensive crisis and increase the mortality rate. This infrequent type of paraganglioma is only recognized through histological examination following surgery. The current study reports the case of a 61-year-old male with urinary bladder paraganglioma. The patient presented with hypertension, which was controlled to within a normal range using diovan and norvasc treatment; in addition, the patient's blood pressure was not altered with urination or postural changes. The patient was not administered an α-blocking agent or a blood volume expander prior to the surgery, and during the partial cystectomy no hypertensive crisis occurred.

Primary paraganglioma of urinary bladder: Case series and review of the literature

Paragangliomas of the urinary bladder (PUB) are uncommon and represent less than 1 per cent of all bladder tumours. They can manifest with classic symptoms and signs similar to those of adrenal pheochromocytoma, but the clinical presentation could be nonspecific. Preoperative diagnosis of this uncommon condition is difficult but important, owing to the potential for catecholamine crisis during surgery. In the present study, we review our experience of four cases of PUB and review the literature on this condition.

Association of urinary bladder paragangliomas with germline mutations in the SDHB and VHL genes

ObjectiveOur primary goal was to examine the clinical characteristics of a series of patients with urinary bladder paragangliomas (UBPGLs), focusing particularly on their genetic backgrounds. Materials and methodsWe analyzed the medical records of patients who presented to the National Institutes of Health with UBPGL from 2000 to 2013 to determine their clinical characteristics and outcomes, biochemical phenotype, tumor size, and genetic background. ResultsOf the 27 patients with UBPGLs who were identified, 17 (63%) had underlying genetic mutations. Overall, 14 (51.9%) patients had a germline mutation in the succinate dehydrogenase subunit B gene (SDHB), and 3 (11.1%) had mutations in the von Hippel-Lindau gene (VHL). Of the 21 patients who had biochemical data available before their first operation, 19 (90.5%) presented with a noradrenergic biochemical phenotype; 7 (33.3%) patients had tumors that also secreted dopamine. In addition, 1 patient (4.8%) had elevated metanephrine levels, and 2 (9.5%) had normal biochemical data. In total, 13 (48.1%) patients in the series were diagnosed with metastatic disease, at either first presentation or follow-up; 6 of these patients (46.1%) had SDHB mutations. ConclusionsUBPGLs typically present with a noradrenergic phenotype and are frequently associated with underlying germline mutations. Patients presenting with these rare neuroendocrine tumors should be screened for these mutations. In addition, patients with UBPGLs should be followed up closely for metastatic development regardless of genetic background, as almost half of the patients in this series presented with metastatic disease and less than half of them had SDHB mutations.

Patients With Urinary Bladder Paragangliomas: A Compiled Case Series From a Literature Review for Clinical Management

Pheochromocytomas are neuroendocrine tumors that arise from chromaffin tissues, typically of the adrenal medulla.1 Approximately 10% are found in extra-adrenal sites along the sympathetic chain, and as such, they are termed as paragangliomas. The urinary bladder is an unusual primary location, accounting for <0.06% of all bladder tumors and <1% of all paraganglionas.2 Typical presentation is related to symptoms of catecholamine excess, such as hypertension, post micturition syndrome, and painless hematuria.

Diuretic 68Ga DOTANOC PET/CT in Imaging of Bladder Paraganglioma

Urinary bladder paragangliomas are exceedingly rare tumors. A 22-year-old woman with bladder paraganglioma underwent Ga DOTANOC PET/CT for proper localization of the primary tumor and to rule out locoregional and distant metastases. Ga DOTANOC-avid bladder mass was detected with no other abnormal site of radiotracer uptake elsewhere. Although radioactive urine can mask urinary paragangliomas, diuretic method can aid tumor detection by Ga DOTANOC PET/CT.

Ganglioneuroma with leiomyomatosis of the urinary bladder: A rare tumor causing frequent micturition and dysuria

We present the case of a 34-year-old female, complaining of frequent micturition and progressive dysuria. By cystoscopy and computerized tomography, we found that she had multiple sub-mucosal protuberant lesions in the entire bladder. After surgical excision, she has thus far showed no further signs of the disease. The postoperative histopathologic examination revealed a ganglioneuroma and leiomyomatosis. Urinary bladder paraganglioma is an unusual tumour; when it is combined with leiomyomatosis, it is extremely rare. We describe the main clinical presentation, diagnostic procedures, and pathologic features. We also review the English literature.

Non-functioning paraganglioma of the urinary bladder: A case report and review of the literature

Paragangliomas are extra-adrenal tumors of the autonomic nervous system and may be found within the skull base, neck, mediastinum and periaortic region. Paragangliomas of the urinary bladder are rare, and non-functioning bladder paraganglioma is even rarer and not easily recognized. Histological examination is often key in leading to a definitive diagnosis. The current report presents a case of a 28-year-old female with urinary bladder paraganglioma. The patient presented with no classical signs and symptoms, and these were only appreciated following histological examination of a transurethral resection specimen that elucidated the correct diagnosis. In the present report, the clinical features, diagnosis, management and pathological observations of paraganglioma of the urinary bladder are discussed.

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.

GATA3 expression in paragangliomas: a pitfall potentially leading to misdiagnosis of urothelial carcinoma

GATA3 is a zinc-finger transcription factor, which is expressed in various normal and neoplastic tissues. Amongst tumors, it labels urothelial carcinoma, collecting duct carcinoma of the kidney, breast carcinoma, lymphoma and, uncommonly, endometrial carcinoma. Few studies have investigated its positivity in various neoplasms that may mimic urothelial neoplasms. In this study, we evaluated GATA3 expression in urinary bladder paragangliomas, which may closely mimic urothelial carcinomas. We retrieved 12 cases of paragangliomas from the urinary bladder and 20 cases of paragangliomas from non-urologic sites using the Hopkins Pathology Data Base system. GATA3 was positive in 10 of the 12 (83%) urinary bladder paragangliomas studied on routine slide sections. Most (6/12) of the staining was diffusely strong (3+) staining, whereas the rest (4/12) that were positive showed mixed intensities (strong 3+ to moderate 2+). The 20 paragangliomas from other sites were constructed into tissue microarrays, wherein three cores from each tumor were taken. Fifteen out of 20 (75%) paragangliomas outside of the bladder were positive for GATA3 staining. Moderate (2+) or strong (3+) staining was seen in 13/20 (65%) of extravesical paragangliomas, ranging from 5 to 100% of the cell labeling (mean 59%, median 60%). In the remaining 7/20 (35%) cases, only weak (2/7) or negative (5/7) immunoreactivity for GATA3 was seen. An additional 15 cases of metastatic paraganglioma from various primary sites were retrieved with 12 of 15 (80%) metastatic paragangliomas staining positively for GATA3. Overall, for paragangliomas, regardless of site, 78.7% were positive for GATA3. Recognition of this finding will aid pathologists in preventing a misdiagnosis of a urothelial tumor based on GATA3 expression, which is critical given the differences in treatment, follow-up and prognosis between bladder paragangliomas and urothelial carcinoma.

Urinary bladder malignant paraganglioma with vertebral metastasis: a case report with literature review

Paraganglioma is a rare neuroendocrine neoplasm observed in patients of all ages, with an estimated incidence of 3/1,000,000 population. It has long been recognized that some cases are familial. The majority of these tumors are benign, and the only absolute criterion for malignancy is the presence of metastases at sites where chromaffin tissue is not usually found. Some tumors show gross local invasion and recurrence, which may indeed kill the patient, but this does not necessarily associate with metastatic potential. Here, we report a case of vertebral metastatic paraganglioma that occurred 19 months after the patient had undergone partial cystectomy for urinary bladder paraganglioma. We believe this to be a rarely reported bone metastasis of paraganglioma arising originally within the urinary bladder. In this report, we also provide a summary of the general characteristics of this disease, together with progress in diagnosis, treatment, and prognosis.

Endoscopic surgical strategy of non-functional urinary bladder paraganglioma in children

Endoscopic surgical strategy of non-functional urinary bladder paraganglioma in children

An Extra-adrenal Pheochromocytoma Presenting as Malignant Hypertension-A Report of two Cases

Malignant hypertension is a complication of hypertension characterized by elevated blood pressure (200mm/140mm Hg), is considered a medical emergency and is rarely secondary to paraganglioma. Malignant hypertension is unique in its relationship to a catecholamine secreting paraganglioma. We present two rare cases of malignant hypertension associated with paraganglioma of tonsil and urinary bladder.

Clinical manifestations of paraganglioma of the urinary bladder

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

A Rare Case of Malignant Urinary Bladder Paraganglioma With Distant Metastases Demonstrated by 18F-FDG PET/CT

Urinary bladder paragangliomas are rare tumors, with malignant ones being still rarer. Demonstration of metastases by imaging is crucial, as histological examination of primary cannot reliably distinguish between benign and malignant tumors. We describe a rare case of a patient with malignant urinary bladder paraganglioma in whom 18F-FDG PET/CT was useful in detection of regional lymph nodal and distant metastases. In addition, 18F-FDG PET/CT suggested an easily accessible site of biopsy to confirm the metastatic disease.

A Rare Case of Incidentally Discovered Urinary Bladder Paraganglioma

A Rare Case of Incidentally Discovered Urinary Bladder Paraganglioma