PCC/PGL是罕见的神经内分泌肿瘤，并能分泌儿茶酚胺。以往的研究表明，SDHB免疫组化可以预测SDHB基因的遗传突变，而SDHB突变与肿瘤进展和转移密切相关。本研究旨在阐明SDHB免疫组化作为PCC/PGL患者肿瘤进展预测标记的潜在作用。我们包括了2002年至2014年在上海交通大学医学院附属瑞金医院被诊断为PCC/PGL的患者进行回顾性分析，并发现SDHB（-）染色患者的预后较差。然后，我们在前瞻性系列中检测了所有肿瘤的SDHB蛋白表达，该系列由我们中心的2015年至2020年患者组成。在回顾性系列中，中位随访时间为167个月，在随访期间，14.4％（38/264）的患者出现转移或复发，8.0％（22/274）的患者死亡。回顾性分析发现，在SDHB（-）组中，66.7％（6/9）的参与者和SDHB（+）组中的15.7％（40/255）的参与者发展为进展性肿瘤（OR：10.75，95％CI：2.72-52.60，P = 0.001），且在调整其他临床病理参数后SDHB（-）与不良预后独立相关（OR：11.68，95％CI：2.58-64.45，P=0.002）。SDHB（-）患者有较短的无病生存期（DFS）和总生存期（OS）（P<0.001），并且多因素Cox比例危险分析显示，SDHB（-）显著与较短的中位DFS相关（HR：6.89，95％CI：2.41-19.70，P<0.001）。在前瞻性系列中，中位随访时间为28个月，4.7％（10/213）的患者出现了转移或复发，0.5％（1/217）的患者死亡。对于前瞻性分析，18.8％（3/16）的SDHB（-）组参与者有进展性肿瘤，与SDHB（+）组的3.6％（7/197）相比（RR：5.28，95％CI：1.51-18.47，P=0.009），即使调整其他临床病理因素，统计学显着性仍然存在（RR：3.35，95％CI：1.20-9.38，P=0.021）。我们的研究结果表明，SDHB（-）肿瘤患者有较高的不良预后可能性，SDHB免疫组化可以作为PCC/PGL患者预后的独立生物标志。版权所有©2023年Su，Yang，Jiang，Xie，Zhong，Wu，Jiang，Zhang，Zhou，Ye，Ning和Wang。

Pheochromocytomas and paragangliomas (PCC/PGL) are rare neuroendocrine tumors and can secrete catecholamine. Previous studies have found that SDHB immunohistochemistry (IHC) can predict SDHB germline gene mutation, and SDHB mutation is closely associated with tumor progression and metastasis. This study aimed to clarify the potential effect of SDHB IHC as a predictive marker for tumor progression in PCC/PGL patients.We included PCC/PGL patients diagnosed in Ruijin Hospital, Shanghai Jiao Tong University School of Medicine from 2002 to 2014 for retrospective analysis and discovered that SDHB (-) staining patients had poorer prognoses. Then we examined SDHB protein expression by IHC on all tumors in the prospective series, which was composed of patients from 2015 to 2020 in our center.In the retrospective series, the median follow-up was 167 months, and during follow-up, 14.4% (38/264) patients developed metastasis or recurrence, and 8.0% (22/274) patients died. Retrospective analysis revealed that 66.7% (6/9) of participants in the SDHB (-) group and 15.7% (40/255) of those in the SDHB (+) group developed progressive tumors (OR: 10.75, 95% CI: 2.72-52.60, P=0.001), and SDHB (-) was independently associated with poor outcomes after adjusting by other clinicopathological parameters (OR: 11.68, 95% CI: 2.58-64.45, P=0.002). SDHB (-) patients had shorter disease-free survival (DFS) and overall survival (OS) (P<0.001) and SDHB (-) was significantly associated with shorter median DFS (HR: 6.89, 95% CI: 2.41-19.70, P<0.001) in multivariate cox proportional hazard analysis. In the prospective series, the median follow-up was 28 months, 4.7% (10/213) patients developed metastasis or recurrence, and 0.5% (1/217) patient died. For the prospective analysis, 18.8% (3/16) of participants in the SDHB (-) group had progressive tumors compared with 3.6% (7/197) in the SDHB (+) group (RR: 5.28, 95% CI: 1.51-18.47, P=0.009), statistical significance remained (RR: 3.35, 95% CI: 1.20-9.38, P=0.021) after adjusting for other clinicopathological factors.Our findings demonstrated patients with SDHB (-) tumors had a higher possibility of poor outcomes, and SDHB IHC can be regarded as an independent biomarker of prognosis in PCC/PGL.Copyright © 2023 Su, Yang, Jiang, Xie, Zhong, Wu, Jiang, Zhang, Zhou, Ye, Ning and Wang.