Clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic extra-adrenal paragangliomas: insights from the largest single-institutional experience.

Clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic extra-adrenal paragangliomas: insights from the largest single-institutional experience.
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DOI:
10.1002/cncr.26577
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发表时间:
2012-06-01
期刊:
影响因子:
6.2
通讯作者:
Jimenez C
Jimenez C
中科院分区:
医学1区
文献类型:
--
作者:
Ayala-Ramirez M;Feng L;Habra MA;Rich T;Dickson PV;Perrier N;Phan A;Waguespack S;Patel S;Jimenez C

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本研究的目的是评估全身化疗对转移性嗜铬细胞瘤或交感神经节细胞瘤患者的临床益处,评估肿瘤大小、血压的缩小和总体生存的改善。我们回顾了在德克萨斯大学MD安德森癌症中心接受化疗的转移性嗜铬细胞瘤-交感性副神经节瘤患者的医疗记录,评估了临床益处和总存活率(OS)。在接受化疗的54名患者中,52名患者的反应是可评估的。17人(33%)在第一次化疗后有反应,定义为血压下降或正常化/降压药物的数量和剂量减少和/或肿瘤体积缩小。应答者的中位OS时间为6.4年(95%可信区间:5.2~16.4),无应答者为3.7年(95%CI:3.0~7.5)。在有同步转移疾病的患者中,化疗开始后1年的阳性反应与总生存期延长的趋势相关(对数等级检验,P值=0.095)。在多因素Cox比例风险模型中,化疗反应对总生存率的影响显著(风险比=0.22,95%可信区间:0.05-1.0;P值=0.05)。所有应答者都接受了达卡巴肼和环磷酰胺的治疗。长春新碱用于14名应答者,阿霉素用于12名应答者。我们不能确定预测化疗反应的临床因素。在大约33%的转移性嗜铬细胞瘤交感神经节瘤患者中,化疗可以缩小肿瘤大小并促进血压控制。这些患者表现出更长的生存时间。
The purpose of this study was to evaluate the clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic paragangliomas by assessing reduction in tumor size, blood pressure, and improvement in overall survival. We retrospectively reviewed the medical records of patients with metastatic pheochromocytomas-sympathetic paragangliomas who had received chemotherapy at The University of Texas MD Anderson Cancer Center Clinical benefit and overall survival (OS) were assessed. Of fifty-four patients treated with chemotherapy, fifty-two were evaluable for response. Seventeen (33%) experienced a response, defined as decreased or normalized blood pressure/decreased number and dosage of antihypertensive medications and/or reduced tumor size after the first chemotherapy regimen. The median OS time was 6.4 years (95 confidence interval (CI): 5.2–16.4) for responders and 3.7 (95% CI: 3.0–7.5) years for non-responders. Of patients who had synchronous metastatic disease, a positive response at 1 year after the start of chemotherapy was associated with a trend toward a longer overall survival (log-rank test, P-value =0.095). In a multivariate Cox proportional hazards model, the effect of response to chemotherapy on overall survival was significant (hazard ratio=0.22, 95% confidence interval: 0.05–1.0; P-value = 0.05). All responders had been treated with dacarbazine and cyclophosphamide. Vincristine was included for 14 responders and doxorubicin was included for 12 responders. We could not identify clinical factors that predicted response to chemotherapy. Chemotherapy may decrease tumor size and facilitate blood pressure control in about 33% of patients with metastatic pheochromocytoma-sympathetic paraganglioma. These patients exhibit a longer survival.
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