Paraganglioma of the Bladder

Bhalani, Sheetal M.; Casalino, David D.; Manvar, Ankur

doi:10.1016/j.juro.2011.04.032

Cited by 20 publications

(18 citation statements)

References 5 publications

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Section: Introductionmentioning

confidence: 99%

“…PGLs can be further classified into sympathetic or parasympathetic types 1–4 . Sympathetic PGLs arise from chromaffin cells distributed along the sympathetic chain and typically secrete catecholamines 4 . Parasympathetic PGLs arise from the ganglia distributed along the parasympathetic chain, are usually non-functional, and are generally localized in the base of the skull and neck 1,5,6 .…”

Section: Introductionmentioning

confidence: 99%

“…Of these sites, the bladder is the most common (79.2%) 10,12 . Regardless, bladder PGLs remain extremely rare, accounting for less than 0.06% of all bladder neoplasms and 6% of paragangliomas 4,9,12–19 . About 17% of bladder PGLs are nonfunctional, while the majority cause paroxysmal symptoms such as headache, diaphoresis, and palpitations 10 .…”

Section: Introductionmentioning

confidence: 99%

“…About 17% of bladder PGLs are nonfunctional, while the majority cause paroxysmal symptoms such as headache, diaphoresis, and palpitations 10 . The triad of hypertension, intermittent painless hematuria, and the aforementioned paroxysmal symptoms upon micturition or sexual activity is considered nearly diagnostic of bladder PGLs and is present in roughly 50% of patients 4,6,10,12 .…”

Section: Introductionmentioning

confidence: 99%

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Genitourinary paraganglioma: Demographic, pathologic, and clinical characteristics in the surveillance, epidemiology, and end results database (2000–2012)

Purnell

Sidana

Maruf

et al. 2017

Urologic Oncology: Seminars and Original Investigations

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Background Extra-adrenal paragangliomas (PGLs) are infrequent, benign, neuroendocrine tumors arising from chromaffin cells of the autonomic nervous system. The majority of PGLs are sporadic but up to 32% are associated with inherited syndromes such as neurofibromatosis type 1, von Hippel-Lindau disease, and familial paraganglioma. While most PGLs develop above the umbilicus, they have been reported in the genitourinary (GU) tract. Due to the paucity of literature on the rates of GU paraganglioma, the objective of our study is to describe the demographic, pathologic, and clinical characteristics of GU PGL, and compare them to non-GU sites of PGL using the Surveillance, Epidemiology, and End Results (SEER) database. Methods The SEER 18 database was utilized to identify all cases of PGL from 2000–2012. Demographic, pathologic, and clinical characteristics were described using chi-square and t-test for categorical and continuous variables, respectively. The Kaplan-Meier method was used to compare overall survival between GU and non-GU PGL. Statistical significance was defined as p < 0.05. All analyses were performed using excel and SAS/Stat version 9.4. Results 299 cases of PGL were retrieved from SEER. 20 (6.7%) of the total PGL arose from the GU tract. The mean age at diagnosis was higher in non-GU than GU PGL (50.4±17.2 vs 40.8±15.6, p=0.026). 83.3% of GU PGLs developed in the bladder, followed by the kidneys/renal pelvis (16.7%), and spermatic cord (2%). Non-GU PGL developed most frequently within the endocrine system (43%). PGL, overall, was more common in men than women, and it was more common in whites than all other races. While 55.5% of GU PGLs were organ confined, only 22.2% of non-GU PGLs were localized at diagnosis. All cases of PGL were treated with surgery. There were 2 cause-specific deaths in the GU PGL groups between 2000 and 2012. The 5-year overall survival was 93.3% for GU PGL versus 65.5% in non-GU PGL (p=0.062). Conclusions Genitourinary PGL remains rare with low incidence (6.7% of all PGL cases) in the US population between 2000 and 2012. Bladder PGL represents just 5% of all PGL. Also, GU-PGL had better overall survival compared to PGL developing outside of the GU tract although the p-value only approached statistical significance. The bladder represents the most common site of involvement and surgery is the mainstay of treatment for GU PGL. Clearer prognostic factors, including tumor grade and stage, are needed to better elucidate PGL management in the future; thus, pooled studies from various institutions with detailed clinical information are needed to delineate these prognostic factors.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Genitourinary paraganglioma: Demographic, pathologic, and clinical characteristics in the surveillance, epidemiology, and end results database (2000–2012)

Purnell

Sidana

Maruf

et al. 2017

Urologic Oncology: Seminars and Original Investigations

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“…Malignancy rates specific to bladder PGLs are reported between 5 -15%, and are higher in hereditary conditions. 4 It is therefore advisable to genetically screen patients presenting with malignant PCCs or PGLs for SDHx mutations. 5 Zimmerman et al was the first to report a case of PGL of the bladder in 1953, and since that time there have been fewer than 200 cases described.…”

Section: 2mentioning

confidence: 99%

Case — Bladder paraganglioma in a pediatric patient

Malhotra

Yan

Tabeshi

et al. 2018

CUAJ

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Initial presentationA 12-year-old boy presented with a two-day history of gross hematuria and vomiting. Further questioning revealed a one-year history of position-induced paroxysmal headaches, polyuria, polydipsia, nocturia, dehydration, and lethargy. He denied any syncopal episodes, palpitations, night sweats, blurry vision, or anxiety attacks. He was noted to have normal pubertal development. An abdominal ultrasound was performed, and showed an 8.1 x 7.5cm bladder mass on the posterior bladder wall with hypervascular components (Figure 1). The patient was brought to the operating theater for cystoscopy, which revealed a large, necrotic mass on the postero-lateral bladder wall. Bladder biopsy was performed. There were significant blood pressure fluctuations during the biopsy. Final pathology reported the mass to be a bladder paraganglioma (PGL).The patient received a two-week course of alpha-adrenergic blockade with Prazosin and Amlodipine for blood pressure control prior to definitive surgery. Echocardiogram did not reveal significant cardiomyopathy. PET/CT scan showed no evidence of metastatic disease, but avid F18-fludeoxyglucose ([F-18]FDG) uptake was noted at the tumor site (Figure 2). ManagementThe patient was brought back to operating theatre for treatment via partial cystectomy. The tumor was located near the right ureteric opening, and a ureteric stent was placed for 6 weeks. Pathology reported the resected PGL had negative surgical margins and no invasion into extravesicular fat. The patient was followed with urine metanephrine and blood pressure measurements every three months for surveillance. Subsequent medical genetics consultation revealed an underlying succinate dehydrogenase B (SDHB) gene mutation. First-degree patient relatives were subsequently investigated for the SDHB mutation.

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