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Bladder

Other tumors

Paraganglioma

Reviewers: Gillian Levy, M.D., Yale Medical Center (see Reviewers page)
Revised: 25 April 2011, 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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Tumor removed by segmental resection of bladder wall; patient had intermittent symptoms of excess catecholamine secretion


Pink erythematous mass


Well circumscribed yellow tumor

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


Anastomosing trabecular pattern focally - nesting pattern was present in other areas


Crush artifact with strands of dark staining tumor cells separating fibromuscular connective tissue


Bundles of smooth muscle of bladder wall are separated by tumor, which has a predominantly nesting or alveolar pattern

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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