Villagrán-García Macarena, Muñiz-Castrillo Sergio, Ciano-Petersen Nicolás Lundahl, Vogrig Alberto, Farina Antonio, Villard Marine, Psimaras Dimitri, Alentorn Agusti, Gonçalves David, Fabien Nicole, Rogemond Véronique, Joubert Bastien, Honnorat Jérôme
French Reference Center on Paraneoplastic Neurological Syndromes and Autoimmune Encephalitis, Hospices Civils de Lyon, Hôpital Neurologique, 59 Boulevard Pinel, 69677, Bron Cedex, France.
MeLiS-UCBL-CNRS UMR 5284-INSERM U1314, Université Claude Bernard, Lyon 1, Lyon, France.
J Neurol. 2023 Jan;270(1):283-299. doi: 10.1007/s00415-022-11356-9. Epub 2022 Sep 1.
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.
Retrospective nationwide cohort chart review study and systematic review of the literature.
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).
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.
副肿瘤性神经系统综合征（PNS）与肾细胞癌和膀胱癌（RCC/BC）之间的联系罕见且不明确。我们的目的是根据更新后的PNS标准，对这些不常见的关联进行临床评估。
全国性回顾性队列图表审查研究及文献系统评价。
排除5例因诊断出同时存在其他恶性肿瘤的患者后，确定了10/18例肾细胞癌患者和8/18例膀胱癌患者。此前共发表了31例病例，从而形成了一个包含27/49例肾细胞癌患者和22/49例膀胱癌患者的总体系列。两种癌症中均以小脑综合征为主（肾细胞癌为10/27，37%；膀胱癌为9/22，41%），其次是9/27（33%）例肾细胞癌患者出现脑炎，5/22（23%）例膀胱癌患者出现脑脊髓炎/感觉神经元病。高危抗体在膀胱癌患者中的检测更为频繁（16/19，84%，而肾细胞癌患者为3/13，23%，p = 0.0009），其中Ri抗体最为常见。应用更新后的PNS标准后，膀胱癌患者在PNS诊断确定性的最高程度（可能、很可能和肯定）方面符合率更高（20/22，91%，而肾细胞癌患者为16/27，59%，p = 0.021）。
在对肾细胞癌或膀胱癌患者进行PNS诊断之前，应始终排除第二种肿瘤。然而，虽然这种关联对大多数肾细胞癌患者来说仍不确定，但对于出现小脑共济失调、脑干脑炎或脑脊髓炎/感觉神经元病且伴有高危抗体的膀胱癌患者，偶然因素的作用更有可能。
