Current peri-operative and surgical management of bladder paraganglioma

Abstract

Introduction: In this article, we provide a narrative review of current peri-operative and surgical management of paraganglioma of the urinary bladder (PUB). Methods: Key articles relating to peri-operative optimisation, intra-operative considerations and post-operative follow-up of patients with PUB were identified for review. Discussion: Although the majority of PUBs are functionally active with potential for catecholamine release, typical catecholaminergic symptoms are often overshadowed by the presence of lower urinary tract symptoms such as painless visible haematuria. Consequently, many cases are diagnosed based on post-operative histopathology, resulting in inferior short- and long-term outcomes. Accurate pre-operative diagnosis is crucial as this informs pre-operative optimisation and choice of surgical approach. Partial cystectomy is the mainstay of treatment, but transurethral resection may be appropriate for non-functional tumours <3 cm. The presence of succinate dehydrogenase ( SDH) subunit B (SDHB) pathogenic variants may prompt upfront radical cystectomy. Long-term outcomes of surgical patients remain incompletely characterised and lifelong follow-up is recommended to exclude tumour recurrence. Conclusion: Patients with PUBs require meticulous peri-operative optimisation under the care of an experienced multidisciplinary team. A pre-operative diagnosis is crucial to ensure optimal outcomes and guide surgical decision-making. Level of evidence: 4