SDHB mutation status and tumor size but not tumor grade are important predictors of clinical outcome in pheochromocytoma and abdominal paraganglioma
SDHB mutation status and tumor size but not tumor grade are important predictors of clinical outcome in pheochromocytoma and abdominal paraganglioma
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DOI：
10.1016/j.surg.2016.05.050
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发表时间：
2017-01-01
期刊：
影响因子：
3.8
通讯作者：
Kebebew, Electron
中科院分区：
文献类型：
--
作者：
Assadipour, Yasmine;Sadowski, Samira M.;Kebebew, Electron
背景。长期以来，人们一直希望有一个分期/预后系统来更好地对嗜铬细胞瘤/副神经节瘤进行分类，因为在SDHB突变的情况下，嗜铬细胞瘤/副神经节瘤具有很强的侵袭性。回顾性分析嗜铬细胞瘤/副神经节瘤患者的临床特点和预后，包括基因检测、肿瘤复发/转移、Ki67/ mib1染色和肿瘤有丝分裂指数。SDHB突变患者出现年龄较轻（33.0岁对49.6岁，P < 0.001），局部复发率和远处转移率增加（分别为47.6%对9.1%,P < 0.001和56.3%对9.1%,P < 0.001），中位无病时间间隔较短（89.8个月，95%可信区间36.0-96.4 vs未达到，P < 0.001）。SDHB突变、最大肿瘤直径和开放手术切除与更高的局部复发率和远处转移率相关（P < 0.006）。SDHB突变和肿瘤直径是局部复发（P = 0.09）、局部复发（P = 0.48、P = 0.066）、转移（P >= 0.22）、无病间期（P >= 0.19）的独立危险因素。SDHB状态和原发肿瘤大小比ki67%或有丝分裂指数更能预测患者预后，应作为任何临床相关的预后评分系统的一部分。
Background. A staging/prognostic system has long been desired to better categorize pheochromocytoma/paraganglioma which can be very aggressive in the setting of SDHB mutations.Methods. A retrospective analysis was conducted of clinical characteristics and outcomes including results of genetic testing, tumor recurrence/metastasis, Ki67/MIB1% staining, and tumor mitotic index in patients with pheochromocytoma/paraganglioma.Results. Patients with SDHB mutation presented at younger age (33.0 years old vs 49.6 years old, P < .001), had increased local recurrence and distant metastases (47.6% vs 9.1 %, P < .001, and 56.3% vs 9.1 %, P < .001, respectively), and lesser median disease-free interval (89.8 months, 95% confidence interval 36.0-96.4 vs not reached, P < .001). SDHB mutation, greatest tumor diameter, and open operative resection were associated with a greater rate of local recurrence and distant metastases (P < .006 each). SDHB mutation and tumor diameter were independent risk factors for local recurrence (P = .09 each), local recurrence (P = .48, P = .066, respectively), metastases (P >= .22 each), or disease-free interval (P >= .19 each).Conclusion. SDHB status and primary tumor size are more predictive of patient outcome than Ki67 % or mitotic index and should be part of any clinically relevant, prognostic scoring system.