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).
CONCLUSIONS: 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.
方法:回顾性全国队列研究和文献系统综述。
结果:由于诊断为另一种并发恶性肿瘤而排除5例患者后,确定了10/18例RCC患者和8/18例BC患者。以前共发表了31例病例,产生27/49RCC和22/49BC患者的总体系列。两种癌症均以小脑综合征为主(10/27,RCC为37%;9/22,BC为41%),其次是9/27(33%)的RCC患者的脑炎和5/22(23%)的BC患者的脑脊髓炎/感觉神经元病变。在BC患者中,高危Abs的检测更为频繁(16/19,84%vs.3/13,碾压混凝土中23%,p=0.0009),Ri抗体是其最常见的。应用更新的PNS标准后,BC患者达到最高程度(可能,可能,并确定)PNS诊断的确定性(20/22,91%与16/27,碾压混凝土中59%,p=0.021)。
结论:在确定RCC或BC患者的PNS诊断之前,应始终排除第二肿瘤。然而,虽然这种关联对于大多数RCC患者来说仍然是可疑的,一个偶然的角色是更可能的BC和高风险的抗体表现为小脑共济失调的患者,脑干脑炎或脑脊髓炎/感觉神经病变。