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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• 作者：Yasmine Assadipour ; MD^a ; ^b ; Samira M. Sadowski ; MD^c ; ^d ; Meghna Alimchandani ; MD^e ; Martha Quezado ; MD^e ; Seth M. Steinberg ; PhD^f ; Naris Nilubol ; MD^c ; Dhaval Patel ; MD^c ; Tamara Prodanov ; MD^g ; Karel Pacak ; MD^g ; Electron Kebebew ; MD^c ; ^{kebebewe@mail.nih.gov}
• 刊名：Surgery
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：161
• 期：1
• 页码：230-239
• 全文大小：1088 K
• 卷排序：161

文摘

A staging/prognostic system has long been desired to better categorize pheochromocytoma/paraganglioma which can be very aggressive in the setting of SDHB mutations.MethodsA 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.ResultsPatients 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 ≤ .04 each) and metastases. Ki67% and mitotic index were not associated with SDHB mutation (P ≥ .09 each), local recurrence (P = .48, P = .066, respectively), metastases (P ≥ .22 each), or disease-free interval (P ≥ .19 each).ConclusionSDHB 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.