Clinical outcome of 50 patients with malignant abdominal paragangliomas and malignant pheochromocytomas
Clinical outcome of 50 patients with malignant abdominal paragangliomas and malignant pheochromocytomas
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DOI：
10.1677/erc.0.0050059
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发表时间：
1998-03-01
影响因子：
3.9
通讯作者：
Vassilopoulou-Sellin, R
中科院分区：
文献类型：
--
作者：
Vassilopoulou-Sellin, R
背景资料：副神经节系统肿瘤是一种独特的，虽然不常见，临床实体的特点是儿茶酚胺分泌过多和血流动力学不稳定，初步病理检查往往不能预测良性与恶性行为。对已知恶性肿瘤患者的临床结果进行分析可能有助于加强所有嗜铬细胞瘤或副神经节瘤患者的初步评估和治疗计划。方法：在德克萨斯大学MD安德森癌症中心，1971年至1995年间诊断出30例恶性腹部副神经节瘤患者和20例恶性嗜铬细胞瘤患者。他们的医疗记录进行了审查，特别注意的临床特点和疾病outcome.Results：在30例副神经节瘤患者中，73%是男性，90%的年龄小于50岁的诊断时。16例患者在初步诊断后0.2至25年仍存活，8例患者在0.8至32年内死于疾病。区域复发和骨转移是最突出的事件。在20例嗜铬细胞瘤患者中，60%为男性，70%在诊断时年龄小于50岁。10名患者在初步诊断后0.8至20年仍存活，而5名患者在1.5至39年内死于疾病。高血压是一个突出的表现特征和区域复发是最常见的治疗failure.Conclusions模式：重要的临床差异区分肾上腺嗜铬细胞瘤从肾上腺外，腹部副神经节瘤。副神经节瘤患者作为一个群体，年轻男性，更可能有恶性病变和更积极的临床过程。恶性嗜铬细胞瘤患者通常表现为高血压，年龄稍大，侵袭性较低。
Background: Tumors of the paraganglionic system represent a distinct, albeit uncommon, clinical entity characterized by catecholamine hypersecretion and hemodynamic instability; initial pathologic examination often cannot predict benign vs malignant behavior. An analysis of the clinical outcome of patients with known malignant tumors may serve to enhance the initial evaluation and therapeutic plan of all patients presenting with pheochromocytoma or paraganglioma.Methods: At the University of Texas M D Anderson Cancer Center, 30 patients with malignant abdominal paraganglioma and 20 patients with malignant pheochromocytoma were diagnosed between 1971 and 1995. Their medical records were reviewed with particular attention to clinical characteristics and disease outcome.Results: Among the 30 patients with paraganglioma, 73% were men, and 90% were younger than 50 years at the time of diagnosis. Sixteen patients have remained alive with persistent disease 0.2 to 25 years after initial diagnosis while eight patients died of their disease within 0.8 to 32 years. Regional recurrence and skeletal metastases were the most prominent events. Among the 20 patients with pheochromocytoma, 60% were men and 70% were younger than 50 years at the time of diagnosis. Ten patients have remained alive with persistent disease 0.8 to 20 years after initial diagnosis while five patients died of their disease within 1.5 to 39 years. Hypertension was a prominent presenting feature and regional recurrence was the most frequent pattern of treatment failure.Conclusions: Important clinical differences distinguish adrenal pheochromocytomas from extraadrenal, abdominal paragangliomas. Patients with paragangliomas are, as a group, younger men, more likely to have malignant lesions and a more aggressive clinical course. Patients with malignant pheochromocytomas usually present with hypertension, are somewhat older, and have less aggressive disease.