scholarly journals MON-907 Silent Presentation of Urinary Bladder Paraganglioma: A Case Report

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Johnathan Kirupakaran ◽  
Giovanna Rodriguez ◽  
Gul Bahtiyar

Abstract Background: Paraganglioma is a rare extraadrenal, neuroendocrine tumor of the sympathetic or parasympathetic ganglia (1). Bladder paraganglioma accounts for 1% of all paragangliomas and 0.05% of all bladder tumors (1,2). Characteristic presentation of this tumor includes hypertensive crisis and postmicturition syncope (3). Clinical Case: 35 year old Middle Eastern man with no pertinent past medical history presented with gross hematuria associated with dysuria, purulent penile discharge, urinary frequency, suprapubic pain, and bilateral flank pain for eight months for which he was given a course of an unknown antibiotic in his home country. Social history revealed smoking half a pack of cigarettes/day for 7 years. Has no family history of cancer. On physical exam he had normal blood pressure with mild tenderness to palpation in suprapubic region. U/A was remarkable for hematuria. Patient was treated for UTI with a course ciprofloxacin without resolution of symptoms. Culture returned negative. CT abdomen/pelvis yielded an approximately 1.9 cm enhancing mural nodule involving the anterior aspect of the bladder. Free metanephrine, nor-metanephrine, plasma catecholamine, and urine vanillylmandelic acid were within normal limits. Cystoscopy and transurethral resection of the tumor (TURBT) was completed; a 5-6mm tumor was visualized and resected. Surgical pathology demonstrated tumor growth into the lamina propria and muscularis propria. Neoplastic cells in the sample were diffusely positive for synaptophysin and chromogranin. S100 protein immunostain highlighted sustentacular cells. The diagnosis of paraganglioma was made based on surgical pathology. Post TURP MRI yielded an approximately 1.7 x 0.9 cm transmural hypervascular enhancing mass lesion involving the anterior aspect of the bladder. Given transmural extension of the tumor, an open partial cystectomy was performed. The tumor was resected successfully with resolution of symptoms. Conclusion: This is an exceedingly rare example of a hormonally silent paraganglioma, mimicking the presentation of a UTI. This case may present the unique consideration of tobacco specific carcinogens as a potential risk factor for the development of bladder paraganglioma. References 1. Benn, DE, Robinson, BG, & Clifton-Bligh, RJ. (2015). “Clinical Manifestations of Paraganglioma Syndromes Types 1-5”. Endocrine-Related Cancer, vol. 22, T91-T103. 2. Loveys, FW, Pushpanathan, C, & Jackman, S. Urinary Bladder Paraganglioma: AIRP Best Cases in Radiologic-Pathologic Correlation”. RadioGraphics, vol 35, no. 5, 1433-1438. 3. Priyadarshi, V, & Pal DK (2015). “Paraganglioma of Urinary Bladder”. Urology Annals, vol 7, no. 3, 402-404.

2018 ◽  
Vol 25 (2) ◽  
pp. 57-62
Author(s):  
Yara A. Fayoumi ◽  
Sarah A. Almagharbi ◽  
Taha A. Abdel-Meguid ◽  
Ahmed Abdelsalam ◽  
Najla M. Alghanmi ◽  
...  

Urinary bladder paragangliomas are rare with a reported incidence of <1% of bladder tumors. We presented a case of a urinary bladder paraganglioma in a 26-year-old male who presented with a painless gross hematuria and hypertensive crisis occurring during his trans-urethral resection. Our diagnosis was based upon patient’s history, abdominal-pelvic computed tomography scan, which revealed a left-sided bladder wall mass 2.26 X 4.4 X 3.28 cm that was obstructing the left distal ureter and causing significant left severe hydroureter and hydronephrosis. Preoperative preparation was done with beta blocker and alpha blocker two weeks prior to surgery. The patient underwent an open surgical partial cystectomy, left nephroureterectomy and, complete external and internal iliac lymphadenectomy. Gross pathological examination of the mass revealed a 3x4.5x4 cm growth originating from the wall of the urinary bladder. Microscopic examination revealed nests invaded into muscularis propria of the urinary bladder, with no evidence of distant metastasis. Immunohistochemical studies showed positive tumor cells staining for chromogranin and synaptophysin while S100 highlighted the surrounding sustentacular cells. During the first 3-month post-operative follow-up period, the patient’s blood pressure was stabilized and the symptoms gradually subsided. Urinary bladder paragangliomas should be considered among the differential diagnosis of bladder tumors.


2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Ryan Findlay ◽  
Angela Rutledge ◽  
Sonja Payne ◽  
Richard Inculet ◽  
Sachin Pandey ◽  
...  

Abstract A 32-year-old male presented with a 4-year history of palpitations, diaphoresis, and orthostatic hypotension. One year prior to presentation, he began experiencing severe, episodic headaches and neck pressure. Neurological work-up included an MRI head, neck, and spine, identifying a left posterior mediastinal lesion adjacent to the T5 vertebral body, approximately 2.4 x 2.0 cm. A community surgical center attempted mass resection via Video -assisted (VATS) approach. Resection was aborted intraoperatively due to a hypertensive crisis following lesion manipulation, with his systolic blood pressure increasing above 325 mmHg. Subsequent evaluation revealed a 24-hour urine norepinephrine of 6117 nmol/d (normal &lt;575) and normetanephrines of 16.6 umol/d (normal &lt;3.4). An MIBG study showed intense tracer avidity in the left paraspinal lesion and minimal uptake in the adrenal glands. A functional paravertebral paraganglioma was diagnosed and the patient was initiated on alpha and beta-blockade in preparation for staged combined treatment. Spinal angiography was performed to define the arterial supply of spinal cord and tumor, which confirmed a hypervascular left thoracic paraspinal tumor. Arterial supply emanated from the left T4 intercostal and left supreme intercostal arteries and a prominent draining vein was present. Trans-arterial particle embolization was performed via both arteries with minimal residual tumor blush present post procedure. Successful VATS excision of the tumor was completed one week after arterial embolization with minimal blood loss. Pathology of the resected tumor revealed an overall intact tumor that stained diffusely positive with synaptophysin and sustentacular cells highlighted by S100 protein with a few foci of coagulative necrosis. Episodes of hypertension (maximum BP 210/80) occurred during embolization and surgical resection with vascular manipulation. These were managed with intraoperative nitroprusside and phentolamine. Peri-procedural plasma metanephrine and cathecholamine levels were collected. Normetanephrine levels (normal &lt;=0.89 nmol/L) were 4.66 before embolization, &gt;6.25 after embolization, 3.70 the day after embolization, 4.10 before VATS, and 0.69 after VATS. Norepinephrine levels (normal 0.8–3.4 nmol/L) were 24.3, 111.7, 61.1, 19.6, and 4 respectively. Genetic testing showed no hereditary predisposition. Discussion: Functional neuroendocrine tumours may be difficult to diagnose due to the wide variety of clinical presentations and must be considered when investigating a vascular mediastinal tumor. In our case, pre-operative embolization did not normalise plasma catecholamine levels, likely from incomplete arterial embolization, but helped reduce intra-operative surgical bleeding risk. Alpha- and beta-blockade should be continued until surgical resection, with dose adjustment as required.


2017 ◽  
pp. 32-34
Author(s):  
S.A. Vozianov ◽  
◽  
M.P. Zakharash ◽  
Yu.M. Zakharash ◽  
N.A. Sevast'yanova ◽  
...  

The objective: increase the effectiveness of diagnosis and treatment of women with a hyperactive bladder, combined neurogenic pathology of the lower urinary tract and distal colon. Patients and methods. The study included 73 women. In assessing the symptoms of clinical manifestations and EMG and UFS data, two groups of patients were identified: the first group – women with increased tone of the neuro-muscular structures of the hyperkinetic type; the second group - women with an increased tone of the neuromuscular structures of the hypokinetic type. In the first group, 37 women were examined, whose mean age was 44.5±2.3 years, and the duration of the disease was 16.7±1.6 months. The second group included 36 women, whose average age was 43.4±2.1 years, and the average duration of the disease was 19.7±1.5 months. Results. The performed treatment showed clinically high efficiency of electrostimulation application depending on the state of the tone of the neuromuscular structures of the lower urinary tract and the distal part of the large intestine, which is confirmed by the data of UFS and EMG. Conclusion. The obtained data allowed to develop and optimize methods of treatment of women with hyperactive urinary bladder with a combined neurogenic pathology of the lower urinary tract and distal division of the large intestine. Key words: neurogenic disorders in urination, intestine neurogenic dysfunction, detrusor, uroflowmetry, electromyography.


2014 ◽  
Vol 54 (6) ◽  
pp. 338
Author(s):  
Lukman Oktadianto ◽  
Risky Vitria Prasetyo ◽  
Ninik Asmaningsih Soemyarso ◽  
Mohammad Sjaifullah Noer

Background Renal involvement during the clinical course ofsystemic lupus erythematosus (SLE) is generally considered to bethe most important factor influencing disease prognosis in termsof morbidity and mortality. Various factors have been reported toinfluence the prognosis of lupus nephritis (LN).Objective To analyze clinical signs and laboratory parameters thatmight serve as predictors associated with mortality in pediatricLN.Methods Retrospectively, medical records of children with LNat Soetomo Hospital from 1998 to 2011 were studied. Diagnosisof SLE was based on Revised American Rheumatism Associationcritera, while patients with clinical manifestations of hypertension,abnormal urinalysis, and serum creatinin > 1 mg/dL wereconsidered as lupus nephritis. Cox proportional hazard modelingwas used to assess for associations of clinical signs and laboratoryparameters with mortality. Kaplan-Meier survival analysis wasused to assess the cumulative survival from the time of diagnosisto the outcome.Results There were 57 children with LN of whom 43 (75%) weregirls. The female-to-male ratio was 3:1. Subjects’ mean age was 10.6(SD 6.87) years. The mean time of observation was 51 (SD 74.54)months and 23 (40%) children died. Age, gender, hypertension,hematuria, proteinuria, and anemia were not significant aspredictors for mortality. However, hypertensive crisis (HR=2.79;95%CI 1.16 to 6.75; P=0.02) and initial glomerular filtration rate(GFR) of <75 mL/min/1.73m2 (HR=3.01; 95%CI 1.23 to 7.34;P=0.01) were significant predictors of mortality in children with LN.The mean survival time of LN with hypertensive crisis and initialGFR <75 mL/min/1.73m2 was 36.9 (SD 12.17) months.Conclusion Hypertensive crisis and GFR <75 mL/min/1.73m2 aresignificant predictors of mortality in children with LN.


2013 ◽  
Vol 137 (10) ◽  
pp. 1337-1341 ◽  
Author(s):  
Melissa L. Stanton ◽  
Li Xiao ◽  
Bogdan A. Czerniak ◽  
Charles C. Guo

Context.—Urothelial tumors are rare in young patients. Because of their rarity, the natural history of the disease in young patients remains poorly understood. Objective.—To understand the pathologic and clinical features of urothelial tumors of the urinary bladder in young patients. Design.—We identified 59 young patients with urothelial tumors of the urinary bladder treated at our institution and analyzed the tumors' pathologic features and the patients' clinical outcomes. Results.—All patients were 30 years or younger, with a mean age of 23.5 years (range, 4–30). Thirty-eight patients (64%) were male, and 21 (36%) were female. Most tumors were noninvasive, papillary urothelial tumors (49 of 59; 83%), including papillary urothelial neoplasms of low malignant potential (7 of 49; 14%), low-grade papillary urothelial carcinomas (38 of 49; 78%), and high-grade papillary urothelial carcinomas (4 of 49; 8%). Only a few (n = 10) of the urothelial tumors were invasive, invading the lamina propria (n = 5; 50%), muscularis propria (n = 4; 40%), or perivesical soft tissue (n = 1; 10%). Clinical follow-up information was available for 41 patients (69%), with a mean follow-up time of 77 months. Of 31 patients with noninvasive papillary urothelial tumors, only 1 patient (3%) later developed an invasive urothelial carcinoma and died of the disease, and 30 of these patients (97%) were alive at the end of follow-up, although 10 (32%) had local tumor recurrences. In the 10 patients with invasive urothelial carcinomas, 3 patients (30%) died of the disease and 5 others (50%) were alive with metastases (the other 2 [20%] were alive with no recurrence). Conclusion.—Urothelial tumors in young patients are mostly noninvasive, papillary carcinomas and have an excellent prognosis; however, a small subset of patients may present with high-grade invasive urothelial carcinomas that result in poor clinical outcomes.


2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Faouzi Mallat ◽  
Wissem Hmida ◽  
Adel Slama ◽  
Faouzi Mosbah

Objectives. Pheochromocytoma of the urinary bladder is an extremely rare tumor that typically presents with a hypertensive crisis during micturition. Preoperatively, it may be misdiagnosed due to nonspecific symptomatology, physical, and radiologic findings.Method. We report a case of unsuspected small pheochromocytoma which was incidentally found by CT scan and confirmed by the histological aspects after transurethral resection in a 63-year-old woman. Here, we have described the clinical presentation, physical findings, laboratory investigations, and treatment provided in our case. We have also included radiological images and histopathology slides with input from both radiologists and pathologists. Surgical management and postoperative follow-up are discussed, as are details of previous published data.Results. After undergoing surgical treatment (transurethral resection), our patient is asymptomatic, with complete resolution of her pathology.Conclusion. Diagnosis is difficult before histopathological examination and should be considered in patients with no risk factors for usual bladder tumor. Our purpose is to raise clinician's awareness for this condition so that they will be more likely to diagnose it. This will facilitate prompt diagnosis and treatment and especially prevent complications due to pheochromocytoma which may be severe.


2019 ◽  
Vol 12 (10) ◽  
pp. e231192 ◽  
Author(s):  
Lalit Kumar ◽  
Anubhav Narwal ◽  
Manoj Kumar ◽  
Seema Kaushal

Primary clear-cell urothelial carcinoma (CCUC) is an uncommon type of urothelial cancer with only 16 cases reported in published literature. Due to the rarity of the tumour, its clinical and prognostic values have not been clearly understood. We present one such rare clinical diagnosis in a 60-year- old man who underwent radical cystectomy (RC) with ileal conduit for urinary bladder cancer. Histopathology showed features of high-grade CCUC infiltrating the muscularis propria. Immunohistochemistry revealed diffuse immunopositivity of pan cytokeratin (CK), GATA3, P40, CK7 but was immunonegative for CD10 and vimentin. Our patient expired 4 months after diagnosis. CCUC has recently been included in the WHO 2016 classification of urothelial tumours. Most of the patients present with poor prognosis. Accurate diagnosis and recognition of this unusual variant are essential for better patient management and prognosis. Early RC seems to be the preferred way of management.


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