Clinical experience over 48 years with pheochromocytoma
Clinical experience over 48 years with pheochromocytoma
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DOI：
10.1097/00000658-199906000-00001
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发表时间：
1999-06-01
影响因子：
9
通讯作者：
Scott, HW
中科院分区：
文献类型：
--
作者：
Goldstein, RE;O'Neill, JA;Scott, HW
目的分析大量嗜铬细胞瘤患者的表现、定位、手术治疗、病理和长期结局。背景数据摘要关于嗜铬细胞瘤存在几个争议领域。虽然许多研究报告肾上腺外嗜铬细胞瘤的恶性率高于肾上腺嗜铬细胞瘤，但前者的患者数量较少，缺乏统计学分析。最近也有争论，是否肿瘤的显微特征可能是预测未来的behavior.Methods，从1950年至1998年，作者观察了104例患者的108个嗜铬细胞瘤。对这些患者的结局进行了前瞻性随访。回顾这些患者的医疗记录，以了解其临床表现、定位、手术治疗、病理学和结局。采用Kaplan-Meier生存分布分析患者生存率。结果本研究包括66例女性患者和38例男性患者。手术时的平均年龄为42.3岁。散发病例占患者的84%;其他16%患有多发性内分泌腺瘤病2型，von Recklinghausen病，von Hippel-Lindau病或Carney综合征。在64个肾上腺肿瘤中，55个最初被认为是良性的，6个具有显微镜下恶性特征，3个具有恶性疾病。患者平均随访12.6年。到目前为止，在另外5名患者（无显微镜下疾病）中发生了恶性疾病（恶性肿瘤的总体发生率为13%）。复发发生在切除术后15年。在26例肾上腺外嗜铬细胞瘤中，14例最初被认为是良性的，8例具有显微镜下恶性特征，4例具有恶性疾病。因此，46%的患者患有恶性疾病或具有恶性特征的肿瘤。患者平均随访11.5年。在1例良性疾病患者和1例恶性特征患者中，发生了恶性疾病（恶性肿瘤的总体发生率为23%）。肾上腺和肾上腺外嗜铬细胞瘤的恶性率差异无统计学意义。肾上腺和肾上腺外嗜铬细胞瘤患者的生存率也相似（p = NS）。结论数据表明肾上腺外嗜铬细胞瘤患者与肾上腺嗜铬细胞瘤患者具有相同的恶性肿瘤风险和相同的总生存率。对这些患者进行终身随访是强制性的。
Objective To analyze the presentation, localization, surgical management, pathology, and long-term outcome of a large series of patients with pheochromocytomas.Summary Background Data There are several areas of controversy pertaining to pheochromocytomas. Although many studies report a higher rate of malignancy for extraadrenal pheochromocytomas than for adrenal pheochromocytomas, the number of patients with the former tumor are small and statistical analysis is lacking. There has also been recent debate as to whether microscopic features of the tumor may be predictive of future behavior.Methods From 1950 to 1998, the authors observed 108 pheochromocytomas in 104 patients. The outcome of these patients has been followed prospectively. The medical records of these patients were reviewed for data on the presentation, localization, surgical management, pathology, and outcome. Patient survival was analyzed using Kaplan-Meier survival distributions.Results This study included 66 female patients and 38 male patients. The average age at surgery was 42.3 years. Sporadic cases accounted for 84% of the patients; the other 16% had multiple endocrine neoplasia type 2, von Recklinghausen's disease, von Hippel-Lindau disease, or Carney's syndrome. Of 64 adrenal tumors, 55 were initially considered benign, 6 had microscopic malignant features, and 3 had malignant disease. Mean patient follow-up was 12.6 years. To date, in five additional patients (none with microscopic disease) malignant disease developed (13% overall rate of malignancy). Recurrence occurred as late as 15 years after resection. Of 26 extraadrenal pheochromocytomas, 14 were initially considered benign, 8 had microscopic malignant features, and 4 had malignant disease. Thus, 46% of patients had either malignant disease or tumors with malignant features. Mean patient follow-up was 11.5 years. In one patient with benign disease and in one patient with malignant features, malignant disease developed (23% overall rate of malignancy). The difference in the rate of malignancy was not statistically significant between adrenal and extraadrenal pheochromocytomas. Patients with adrenal and extraadrenal pheochromocytomas also had similar rates of survival (p = NS).Conclusions The data suggest that patients with extraadrenal pheochromocytomas have the same risk of malignancy and the same overall survival as patients with adrenal pheochromocytomas. Lifelong follow-up of these patients is mandatory.