Case: 225

Paraganglioma Of The Bladder

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History: 73 yo female with "cervical lesion".
Solution: A paraganglioma is defined as a rare neuroendocrine tumor arising from paraganglionic tissue found between the base of the skull and floor of the pelvis. If it arises from the adrenal gland, it is called a pheochromocytoma and if it arises from an extra-adrenal location, it is called a paraganglioma. They can be associated with syndromes and have multiple sites of disease (approx 20%) and they may be hormonally active with the associated symptoms. Malignant transformation occurs in approx 2-10%. Paraganglioma of the bladder is rare, accounting for 0.1% of bladder tumors. Can be associated with hematuria, persistent hypertension and in occasionally, the "pathognomonic" finding of micturition induced syncope. They are most frequently seen at the dome or trigone of the bladder. The imaging findings are typically of a hypervascular tumor, generally localized, but can have metastatic disease. The findings that can be clues that this is not a TCC are the hypervascular nature of the tumor, lack of calcification, lack of obstruction of the left ureteral orifice despite the close proximity of the tumor (TCC tends to be more infiltrative while this tumor is very focal). Note that the history is very unhelpful in this case and actually at least 1/3 of patients with paraganglioma of the bladder will have no associated symptoms. The interpreting radiologist initially tried to tie this lesion into the cervix, ignoring the fact that there was no association with the cervix. Do not be too swayed by history...

Questions

The primary abnormality involves the:
  • Bones
  • Ureter
  • Cervix
  • Bladder
  • Colon
  • Uterus
The treatment of this disease is surgical.
True
What is the "classic" history for this disease and what is the diagnosis?
Micturition syncope. Bladder paraganglioma.