Metanephrine normal, DOTATATE negative paraganglioma of the urinary bladder

A woman in her 30s presented with recurrent haematuria and dysuria for 1 year. Imaging revealed a 10 cm pedunculated mass in the bladder, with histopathology confirming paraganglioma. Initial management involved a transurethral biopsy of the tumour, followed by partial cystectomy with ureteric reimplantation. Six months later, she developed lower abdominal pain, and imaging revealed tumour recurrence. A second exploratory laparotomy was performed, confirming recurrent paraganglioma, for which wide local excision with adequate margins was done after a multidisciplinary team discussion comprising urologists, oncologists, radiologists and pathologists. Postoperative recovery was uneventful. Given the recurrent nature of the tumour, long-term follow-up with MRI and Gallium-68 DOTATATE PET-CT was recommended. 6 months later, the patient again developed a second recurrence, and she was advised pelvic exenteration after multidisciplinary tumour discussion. The patient party was not willing to consider this procedure, considering the high morbidity and mortality risk associated with the procedure. She succumbed to death soon after. Bladder paragangliomas are rare neuroendocrine tumours requiring early diagnosis and surgical intervention to prevent a recurrence. This case highlights the importance of histopathology, immunohistochemistry and long-term surveillance in managing bladder paragangliomas to optimise patient outcomes.