Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Etiology | Clinical features | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Differential diagnosis | Additional referencesCite this page: Levy G. Paraganglioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/bladderparaganglioma.html. Accessed October 1st, 2023.
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
- 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
- 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)
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
Positive stains
- Chromogranin, synaptophysin, neuron specific enolase
- S100: sustentacular cells
- GATA3: important pitfall as this overlaps with urothelial carcinoma
Electron microscopy description
- Neurosecretory granules
Molecular / cytogenetics description
- Often not diploid (Hum Pathol 1991;22:1162)
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