{Reference Type}: Systematic Review
{Title}: The association between paraneoplastic neurological syndromes (PNS) and urothelial carcinoma - A review of the literature.
{Author}: Otis SU;Banna GL;Maniam A;
{Journal}: Crit Rev Oncol Hematol
{Volume}: 196
{Issue}: 0
{Year}: 2024 Apr 5
{Factor}: 6.625
{DOI}: 10.1016/j.critrevonc.2024.104314
{Abstract}: Paraneoplastic neurological syndromes (PNS) are rare neurological disorders arising from malignancy-triggered autoimmunity, yet their association with urothelial carcinoma remains unclear. This systematic review intends to explore any connection, alongside patient/clinical features and management. A literature search identified 25 cases of bladder and upper tract carcinoma linked to PNS. Overall, while infrequent, a meaningful association between PNS and urothelial carcinoma was found in that 84% of cases met a 'possible'-or-'higher-likelihood' PNS diagnosis. Most cases presented with high-risk PNS phenotypes, predominantly cerebellar syndromes and encephalomyelitis/sensory neuronopathy, ∼17 months within cancer diagnosis/recurrence. Review findings suggest a female preponderance in suspected PNS despite higher male incidence of urothelial cancer. Main treatments consisted of surgery alongside chemotherapy or immunotherapeutics (IVIG and/or corticosteroids), which improved symptoms for a slight majority (60%). Ultimately, while common PNS-associated neoplasms should always first be excluded in suspected PNS, in the absence of alternative causes, urothelial carcinomas do merit clinical consideration.