琥珀酸脱氢酶B（SDHB）突变状态和肿瘤大小而非肿瘤分级是嗜铬细胞瘤和腹部副神经节瘤临床预后的重要预测指标。

SDHB mutation status and tumor size but not tumor grade are important predictors of clinical outcome in pheochromocytoma and abdominal paraganglioma.

作者信息

Assadipour Yasmine, Sadowski Samira M, Alimchandani Meghna, Quezado Martha, Steinberg Seth M, Nilubol Naris, Patel Dhaval, Prodanov Tamara, Pacak Karel, Kebebew Electron

机构信息

Surgery Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD; Department of Surgery, George Washington University Hospital, Washington, DC.

Endocrine Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD; Thoracic and Endocrine Surgery, University Hospitals of Geneva, Geneva, Switzerland.

出版信息

Surgery. 2017 Jan;161(1):230-239. doi: 10.1016/j.surg.2016.05.050. Epub 2016 Nov 10.

DOI:10.1016/j.surg.2016.05.050

PMID:27839933

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5164946/

Abstract

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.

摘要

背景

长期以来，人们一直期望有一种分期/预后系统，以便更好地对嗜铬细胞瘤/副神经节瘤进行分类，这类肿瘤在存在SDHB突变的情况下可能具有很强的侵袭性。

方法

对嗜铬细胞瘤/副神经节瘤患者的临床特征和预后进行回顾性分析，包括基因检测结果、肿瘤复发/转移、Ki67/MIB1%染色以及肿瘤有丝分裂指数。

结果

SDHB突变患者发病年龄较轻（33.0岁对49.6岁，P <.001），局部复发和远处转移增加（分别为47.6%对9.1%，P <.001，以及56.3%对9.1%，P <.001），无病间隔时间中位数较短（89.8个月，95%置信区间36.0 - 96.4对未达到，P <.001）。SDHB突变、最大肿瘤直径和开放手术切除与局部复发和远处转移率较高相关（各P <.006）。SDHB突变和肿瘤直径是局部复发（各P≤.04）和转移的独立危险因素。Ki67%和有丝分裂指数与SDHB突变（各P≥.09）、局部复发（分别为P =.48，P =.066）、转移（各P≥.22）或无病间隔时间（各P≥.19）无关。