Other tumors

Author: Gillian Levy, M.D. (see Authors page)

Revised: 9 August 2016, last major update December 2014

Copyright: (c) 2003-2016,, Inc.

PubMed Search: Bladder paraganglioma [title]
Cite this page: Paraganglioma. website. Accessed October 22nd, 2016.
Definition / General
  • Tumor derived from paraganglion cells, similar to counterpart at other sites
  • Also called extra-adrenal pheochromocytoma
  • 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
  • All sites in bladder; at any level of bladder wall
  • Muscularis propria is the most common location
  • Derives from paraganglion cells in bladder wall
Clinical Features
  • 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
  • High stage tumors (pT3) have poorer outcomes (Cancer 2000;88:844)
  • Distant metastasis are rare and can occur many years later
Case Reports
  • 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
  • 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
  • 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

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S100+ sustentacular cells

Images hosted on Flickr:
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40 year old woman with bladder tumor

Positive Stains
Negative Stains
  • Keratin
Electron Microscopy Description
  • Neurosecretory granules
Molecular / Cytogenetics Description
Differential Diagnosis
  • 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