American Urological Association - Paraganglioma

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Home Education Educational Programs E-Learning Pathology for Urologists Urinary Bladder Mesenchymal and Other Tumors Paraganglioma

Paraganglioma

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  • Also known as pheochromocytoma of bladder, and is thought to arise from paraganglion tissue within the bladder.
  • Most common presenting symptom is hematuria, although the pathognomonic finding is paroxysmal hypertension with micturition.
  • Gross features: intramural nodules <5 cm in size, usually with intact overlying mucosa (image A).

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  • Histology:
    • Essentially look like paragangliomas elsewhere (think of pheochromocytoma in adrenal gland).
    • Nested growth ("zellballen") of cells with abundant eosinophilic cytoplasm and indistinct cytoplasmic borders (image B) & (image C).
    • Mitoses are rare and necrosis is absent
    • Nuclear atypia may be seen and does not imply a worse prognosis.
  • Immunohistochemistry: positive for neuroendocrine markers synaptophysin (image D) and chromogranin; S100 will stain intermingled sustentacular cells.
  • Majority is benign (90%), but local recurrence may occur with incomplete excision.

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