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  • SDHB mutation status and tu...
    Assadipour, Yasmine, MD; Sadowski, Samira M., MD; Alimchandani, Meghna, MD; Quezado, Martha, MD; Steinberg, Seth M., PhD; Nilubol, Naris, MD; Patel, Dhaval, MD; Prodanov, Tamara, MD; Pacak, Karel, MD; Kebebew, Electron, MD

    Surgery, 01/2017, Letnik: 161, Številka: 1
    Journal Article

    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  ≤ .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). 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.