Bladder & ureters
Other tumors
Paraganglioma


Topic Completed: 1 December 2014

Revised: 12 September 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: Bladder paraganglioma [title]

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Cite this page: Levy G. Paraganglioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/bladderparaganglioma.html. Accessed October 13th, 2019.
Definition / general
  • Tumor derived from paraganglion cells, similar to counterpart at other sites
Terminology
  • Also called extra-adrenal pheochromocytoma
Epidemiology
  • 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
  • All sites in bladder; at any level of bladder wall
  • Muscularis propria is the most common location
Etiology
  • 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
Treatment
  • TUR (transurethral resection), wedge resection or partial cystectomy
  • Malignant paraganglioma: radical cystectomy with removal of metastasis if possible
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

Contributed by Debra Zynger, M.D.
Missing Image

Bladder paraganglioma

Microscopic (histologic) 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
Microscopic (histologic) images

Contributed by Debra Zynger, M.D.

Nested architecture

Amphophilic cytoplasm

Pseudopapillae

Cellular spindling


Mitotic activity

Muscularis propria invasion

Synaptophysin

GATA3



Contributed by Professor David Cohen, Israel

40 year old woman with bladder tumor


ChromograninA

Synaptophysin

NSE

S100

HMB45

Positive stains
Negative stains
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
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