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

Henderson, Sean; Kearns, Patrick J.; Tong, Ching Man Carmen; Reddy, Madhumitha; Khurgin, Jacob; Bickell, Michael; Miick, Robert; Ginsberg, Phillip C.; Metro, Michael J.

doi:10.1016/j.urology.2014.11.006

Cited by 19 publications

(18 citation statements)

References 20 publications

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“…Although 12% arise in the thorax (with 2% being associated with the heart), the majority of sympathetic paragangliomas occur below the diaphragm, particularly in close proximity to the adrenal gland (42%), organ of Zuckerkandl (28%) and bladder (10%) . Unlike their predominantly head and neck parasympathetic counterparts there is an equal gender distribution, with the most common age at presentation being the fifth decade but with a younger age of onset in hereditary disease .…”

Section: Incidence and Locationmentioning

confidence: 99%

Pathology and genetics of phaeochromocytoma and paraganglioma

et al. 2017

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Phaeochromocytoma and paraganglioma (PHEO/PGL) are rare tumours with an estimated annual incidence of 3 per million. Advances in molecular understanding have led to the recognition that at least 30–40% arise in the setting of hereditary disease. Germline mutations in the succinate dehydrogenase genes SDHA, SDHB, SDHC, SDHD and SDHAF2 are the most prevalent of the more than 19 hereditary genetic abnormalities which have been reported. It is therefore recommended that, depending on local resources and availability, at least some degree of genetic testing should be offered to all PHEO/PGL patients, including those with clinically sporadic disease. It is now accepted that that all PHEO/PGL have some metastatic potential; therefore, concepts of benign and malignant PHEO/PGL have no meaning and have been replaced by a risk stratification approach. Although there is broad acceptance that certain features, including high proliferative activity, invasive growth, increased cellularity, large tumour nests and comedonecrosis, are associated with an increased risk of metastasis, it remains difficult to predict the clinical behaviour of individual tumours and no single risk stratification scheme is endorsed or in widespread use. In this review, we provide an update on advances in the pathology and genetics of PHEO/PGL with an emphasis on the changes introduced in the WHO 2017 classification of endocrine neoplasia relevant to practising surgical pathologists.

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Section: Incidence and Locationmentioning

confidence: 99%

Pathology and genetics of phaeochromocytoma and paraganglioma

et al. 2017

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“…Bladder paragangliomas (PGLs) are very rare neuroendocrine tumors. Since the description of the first case in 1953, its incidence is less than 0.05% of all bladder tumors and less than 1% of all pheochromocytomas [1,2]. Among all genitourinary PGLs, the bladder is usually involved (79.2%) [1].…”

Section: Introductionmentioning

confidence: 99%

“…Among all genitourinary PGLs, the bladder is usually involved (79.2%) [1]. This catecholamine-producing neoplasm arises from the ganglion cell in the bladder wall, most commonly on the trigone, with an average tumor size of 3.9 cm [1,2]. They have uncertain malignant potential with unpredictable depth and behavior, demonstrating extravesical invasion in up to 50% of cases.…”

Section: Introductionmentioning

confidence: 99%

“…How this entity can come about remains unclear, but it may have an inherited genetic susceptibility in up to 50% of cases [3]. It occurs in all age groups but more often in patients aged 20-40 years with no sex predominance [1,2]. Most cases present with a well-defined set of symptoms related to excessive catecholamine release during bladder overdistension and/or micturition, including hypertensive crises, headache, palpitations, sweating, hot flushes that may be accompanied by painless hematuria, post micturition hypotension, or syncope [1][2][3].…”

Section: Introductionmentioning

confidence: 99%

“…It occurs in all age groups but more often in patients aged 20-40 years with no sex predominance [1,2]. Most cases present with a well-defined set of symptoms related to excessive catecholamine release during bladder overdistension and/or micturition, including hypertensive crises, headache, palpitations, sweating, hot flushes that may be accompanied by painless hematuria, post micturition hypotension, or syncope [1][2][3]. A high index of clinical suspicion should lead to a measurement of 24-hour urine and/or plasma fractionated catecholamines and metanephrines.…”

Section: Introductionmentioning

confidence: 99%

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Functional Bladder Paraganglioma as an Incidental Finding During Infertility Workup

Hajji¹,

Benazzouz²,

Hammoune³

et al. 2021

Cureus

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Bladder paragangliomas (PGLs) are extremely rare catecholamine-producing neuroendocrine tumors. They arise more frequently in the trigone and have unpredictable depth and behavior. Most cases typically present with a well-defined set of symptoms triggered by micturition or bladder overdistension. Besides long-term follow-up, they are usually managed by either transurethral resection (TUR) or partial cystectomy (PC). However, about 25% of all documented cases do not manifest clinically, raising both diagnosis and management challenges. This report describes an unusual case of a misdiagnosed, functional PGL arising on the bladder dome, which was fortuitously detected in a 21-year-old female during a fertility workup. Owing to its hypervascular nature and submucosal location, bladder PGL was suspected on ultrasound and CT findings and successfully diagnosed before surgery through biochemical confirmation. It was managed by cystoscopy-guided laparoscopic partial cystectomy (LPC) with good oncological and urinary outcomes, as well as no compromise of her fertility potential. To our best knowledge, this is the first case to be incidentally detected on transvaginal ultrasound during evaluation for infertility. This case also stresses the importance of considering PGL in the differential diagnosis of atypical bladder tumors, as well as conservative management through simultaneous laparoscopy and cystoscopy, when approaching young patients with large functional PGL.

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