Recurrent paraganglioma of the urinary bladder 26 years after initial treatment: a case report

Pheochromocytomas and paragangliomas (PPGLs) have malignant potential, and long-term follow-up of patients with PPGLs is recommended by current guidelines. Here, we report a case of recurrent paraganglioma (PGL) of the urinary bladder 26 years after initial treatment.

A 58-year-old woman with a history of partial cystectomy for PGL of the urinary bladder 26 years earlier presented with palpitations, headache during urination, and left inguinal pain. Postoperative follow-up had been discontinued. Imaging studies showed an intravesical mass and multiple masses in the Retzius space and left external iliac region. These masses showed significant uptake on ¹²³I-metaiodobenzylguanidine and ¹⁸F-fluorodeoxyglucose positron emission tomography/computed tomography. Plasma norepinephrine and urinary-free normetanephrine levels were elevated. She underwent partial cystectomy and resection of the masses in the Retzius space and left external iliac region. The histological features were consistent with those of PGL. At 18 months of postoperative follow-up, the random spot urinary-free normetanephrine-to-creatinine and metanephrine-to-creatinine ratios remained normal, with no recurrent disease.

The discontinuation of lifelong surveillance in our patient may have led to a delayed diagnosis of recurrence. Since the mainstay of treatment for PPGL is surgery, appropriate lifelong follow-up contributes to the early detection of recurrence, thereby increasing the likelihood of prompt and complete resection.

Owing to the possibility of late PGL recurrence, lifelong follow-up is required for all patients with PGL.