Malignant paraganglioma of the urinary bladder in a 45-year-old woman.

Paragangliomas are neuroendocrine tumors arising from the extra-adrenal sympathetic or parasympathetic nervous system,1 either from the chromaffin-positive paraganglionic tissue or the chromaffin-negative glomus cells2 derived from the embryonic neural crest. They are localized in either the head and neck, thorax, or abdominal area, and they are usually benign but can occasionally show malignant behavior.3 Although all paragangliomas contain neurosecretory granules, a few secrete clinically significant levels of catecholamines (functional paragangliomas)4 and can cause symptoms of sympathetic surge, including hypertension, anxiety, headaches, diaphoresis, and palpitations. Many paragangliomas are sporadic, but a few cases of familial disease—associated with mutations in the succinyl dehydrogenase genes and multiple endocrine neoplasia 2 syndromes—have been reported.5 Paraganglioma of the urinary bladder is rare, accounting for approximately 0.06% of all bladder tumors.6