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.
Critical reviews in oncology/hematology. 2024 Mar 05 [Epub]
Sarafina Urenna Otis, Giuseppe Luigi Banna, Akash Maniam
Medical Sciences Division, University of Oxford, John Radcliffe Hospital, Oxford, England OX3 9DU, UK., Portsmouth Hospitals University NHS Trust, Portsmouth, England PO6 3LY, UK; Faculty of Science and Health, School of Pharmacy and Biomedical Sciences, University of Portsmouth, Portsmouth, England PO1 2UP, UK., Portsmouth Hospitals University NHS Trust, Portsmouth, England PO6 3LY, UK; Caribbean Cancer Research Institute, Chanka Trace El Socorro South, Trinidad and Tobago. Electronic address: .