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Bladder

Other tumors

Paraganglioma


Reviewers: Gillian Levy, M.D., Yale Medical Center (see Reviewers page)
Revised: 31 March 2014, last major update April 2011
Copyright: (c) 2003-2011, PathologyOutlines.com, Inc.

Definition
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● Tumor derived from paraganglion cells, similar to counterpart at other sites

Terminology
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● Also called extra-adrenal pheochromocytoma

Epidemiology
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● Uncommon in bladder (0.06% to 0.1% of all bladder tumors)
● Median age 41 years vs. 70 years for urothelial carcinoma
● Often young women
● Age range 10-88 years
● Male to female ratio 2:3

Sites
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● All sites in bladder; at any level of bladder wall
● Muscularis propria is the most common location

Etiology
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● Derives from paraganglion cells in bladder wall

Clinical features
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● Many cases are hereditary and associated with familial paraganglioma, neurofibromatosis Type 1, von Hippel-Lindau disease, Carney triad or rarely MEN2 (Ann N Y Acad Sci 2006;1073:21)
● 50% have “micturition attack” during urination with full bladder, consisting of bursting headache, pounding sensation, anxiety, sweating, syncope, hypertension or hematuria
● 50% have positive urinary VMA
● Malignant behavior in 5-15%; cannot be predicted from histology
● 50% have hematuria

Prognostic factors
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● High stage tumors (pT3) have poorer outcomes (Cancer 2000;88:844)
● Distant metastasis are rare and can occur many years later

Case reports
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● 25 year old man with tumor exhibiting malignant features (Internet Journal of Surgery 2008;15(1))
● 55 year old man (Internet Journal of Urology 2009;6(1))
● 64 year old man with composite paraganglioma-ganglioneuroma (Can Urol Assoc J 2009;3:E45)

Treatment
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● TUR (transurethral resection), wedge resection or partial cystectomy
● Malignant paraganglioma: radical cystectomy with removal of metastasis if possible

Clinical images
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Operative photo shows submucosal mass

Gross description (Macroscopy)
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● Usually single, small (2-3 mm), lobulated, well circumscribed, anywhere in bladder (not just base)
● Positive chromaffin reaction (tumor turns dark brown after placement in Zenker’s fixative)

Gross images
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Pink erythematous mass

Micro description (Histopathology)
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● Resembles paragangliomas elsewhere, with zellballen (diffuse patterns of large polygonal cells in clusters separated by a delicate fibrovascular stroma); cells have abundant eosinophilic granular cytoplasm, fairly uniform nuclei with smooth chromatin
● Frequently involves muscularis propria but without a desmoplastic reaction
● Occasionally has degenerative type nuclei
● Rarely has clear cells, tumor necrosis or mitotic figures
● May be oncocytic or mixed with ganglioneuroma

Micro images
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Large polygonal cells surrounded by fibrovascular stroma, with occasional nuclear degenerative changes


S100+ sustentacular cells




40 year old woman with bladder tumor

Positive stains
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● Chromogranin, synaptophysin, neuron specific enolase
● S100+ sustentacular cells

Negative stains
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● Keratin

Electron microscopy description
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● Neurosecretory granules

Molecular / cytogenetic description
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● Often not diploid (Hum Pathol 1991;22:1162)

Differential diagnosis
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Carcinoid tumor: may have focal nesting pattern, keratin+
Melanoma: usually not a defined nesting pattern; is spindled or epithelioid, has prominent nucleoli; S100+, HMB45+, MelanA+
Normal paraganglia
Urothelial carcinoma: may have nesting pattern, but more atypia; lacks prominent vascular network of paraganglioma; keratin+, neuroendocrine markers negative (Am J Surg Pathol 2004;28:94), usually associated with carcinoma in situ or noninvasive papillary carcinoma

Additional references
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eMedicine

End of Bladder > Other tumors > Paraganglioma


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