Macarena Villagrán-García1,2, Sergio Muñiz-Castrillo1,2, Nicolás Lundahl Ciano-Petersen1,2,3,4, Alberto Vogrig5, Antonio Farina1,2, Marine Villard1, Dimitri Psimaras6,7, Agusti Alentorn6,7, David Gonçalves8, Nicole Fabien8, Véronique Rogemond1, Bastien Joubert1,2, Jérôme Honnorat9,10. 1. French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, Hôpital Neurologique, 59 Boulevard Pinel, 69677, Bron Cedex, France. 2. MeLiS-UCBL-CNRS UMR 5284-INSERM U1314, Université Claude Bernard, Lyon 1, Lyon, France. 3. Instituto de Investigación Biomédica de Málaga-IBIMA, Málaga, Spain. 4. Red Andaluza de Investigación Clínica y Traslacional en Neurología (NeuroRECA), Málaga, Spain. 5. Clinical Neurology Unit, Santa Maria della Misericordia University Hospital, Udine, Italy. 6. Neurology 2 Department Mazarin, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, 75013, Paris, France. 7. Sorbonne Université, Inserm, CNRS, Paris Brain Institute, Institut du Cerveau et de la Moelle Épinière, ICM, 75013, Paris, France. 8. Immunology Department, Hôpital Lyon Sud, Hospices Civils de Lyon, Pierre-Bénite, France. 9. French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, Hôpital Neurologique, 59 Boulevard Pinel, 69677, Bron Cedex, France. jerome.honnorat@chu-lyon.fr. 10. MeLiS-UCBL-CNRS UMR 5284-INSERM U1314, Université Claude Bernard, Lyon 1, Lyon, France. jerome.honnorat@chu-lyon.fr.
Abstract
BACKGROUND: The link between paraneoplastic neurological syndromes (PNS) and renal cell and bladder cancer (RCC/BC) is rare and uncertain. Our aim was to clinically evaluate, in light of the updated PNS criteria, these uncommon associations. METHODS: Retrospective nationwide cohort chart review study and systematic review of the literature. RESULTS: After excluding 5 patients due to the diagnosis of another co-occurrent malignancy, 10/18 patients with RCC and 8/18 patients with BC were identified. A total of 31 cases were previously published, yielding an overall series of 27/49 RCC and 22/49 BC patients. There was a predominance of cerebellar syndromes in both cancers (10/27, 37% for RCC; 9/22, 41% for BC), followed by encephalitis in 9/27 (33%) patients with RCC and encephalomyelitis/sensory neuronopathy in 5/22 (23%) patients with BC. The detection of high-risk Abs was more frequent among BC patients (16/19, 84% vs. 3/13, 23% in RCC, p = 0.0009), Ri antibodies being the most frequent thereof. After applying the updated PNS criteria, patients with BC met highest degrees (possible, probable, and definite) of certainty for PNS diagnosis (20/22, 91% vs. 16/27, 59% in RCC, p = 0.021). CONCLUSION: A second neoplasm should always be ruled out before establishing the diagnosis of PNS in patients with RCC or BC. However, while this association remains dubious for most patients with RCC, a casual role is more probable in patients with BC and high-risk antibodies presenting with cerebellar ataxia, brainstem encephalitis or encephalomyelitis/sensory neuronopathy.
BACKGROUND: The link between paraneoplastic neurological syndromes (PNS) and renal cell and bladder cancer (RCC/BC) is rare and uncertain. Our aim was to clinically evaluate, in light of the updated PNS criteria, these uncommon associations. METHODS: Retrospective nationwide cohort chart review study and systematic review of the literature. RESULTS: After excluding 5 patients due to the diagnosis of another co-occurrent malignancy, 10/18 patients with RCC and 8/18 patients with BC were identified. A total of 31 cases were previously published, yielding an overall series of 27/49 RCC and 22/49 BC patients. There was a predominance of cerebellar syndromes in both cancers (10/27, 37% for RCC; 9/22, 41% for BC), followed by encephalitis in 9/27 (33%) patients with RCC and encephalomyelitis/sensory neuronopathy in 5/22 (23%) patients with BC. The detection of high-risk Abs was more frequent among BC patients (16/19, 84% vs. 3/13, 23% in RCC, p = 0.0009), Ri antibodies being the most frequent thereof. After applying the updated PNS criteria, patients with BC met highest degrees (possible, probable, and definite) of certainty for PNS diagnosis (20/22, 91% vs. 16/27, 59% in RCC, p = 0.021). CONCLUSION: A second neoplasm should always be ruled out before establishing the diagnosis of PNS in patients with RCC or BC. However, while this association remains dubious for most patients with RCC, a casual role is more probable in patients with BC and high-risk antibodies presenting with cerebellar ataxia, brainstem encephalitis or encephalomyelitis/sensory neuronopathy.
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