John H, Ziegler W H, Hauri D, Jaeger P
Urologic Clinic, University Hospital Zürich, Switzerland.
Urology. 1999 Apr;53(4):679-83. doi: 10.1016/s0090-4295(98)00612-8.
The presence of metastatic lesions is the only acceptable fact to confirm malignant pheochromocytoma. Patients with malignant pheochromocytomas, however, have a very poor survival rate. The aim of our study was to postulate predictive values for malignant pheochromocytomas.
We evaluated symptoms, diagnostic modalities, treatment, and long-term follow-up of 86 patients with 85 benign and 10 malignant pheochromocytomas. Parameters from the benign were compared with those of the malignant pheochromocytomas.
Preoperative 24-hour urinary dopamine was in the normal range for benign pheochromocytomas but increased in malignant pheochromocytomas (P<0.0001). Vanillylmandelic acid was elevated in both benign and malignant pheochromocytomas but higher in malignant than in benign tumors (P = 0.01). No differences could be shown in urinary epinephrine and norepinephrine samplings. Tumor location was divided into 77 adrenal (81%) and 18 extra-adrenal (19%) sites. Malignant pheochromocytomas were located more often at extra-adrenal sites (P = 0.03). There was no increased incidence of malignancy in patients with familial bilateral pheochromocytomas or multiple endocrine neoplasia. Tumors greater than 80 g in weight corresponded to malignancy (P<0.0001). Dopamine tumor concentration was higher in malignant than in benign pheochromocytomas (P = 0.01). Persistent arterial hypertension occurred in 9 (13%) of 72 benign and 6 (60%) of 10 malignant pheochromocytomas (P = 0.001). The 10-year survival rate was 94% for benign pheochromocytomas. All patients with malignant pheochromocytomas died within this period (P = 0.0001).
High preoperative 24-hour urinary dopamine levels, extra-adrenal tumor location, high tumor weight, elevated tumor dopamine concentration, and postoperative persistent arterial hypertension are all factors that increase the likelihood of malignant pheochromocytoma. Patients with these characteristics should have more frequent follow-up evaluations to identify malignancy at earlier states.
存在转移病灶是确诊恶性嗜铬细胞瘤的唯一可接受依据。然而，恶性嗜铬细胞瘤患者的生存率极低。我们研究的目的是推测恶性嗜铬细胞瘤的预测指标。
我们评估了86例患有85个良性和10个恶性嗜铬细胞瘤患者的症状、诊断方法、治疗及长期随访情况。将良性嗜铬细胞瘤的各项参数与恶性嗜铬细胞瘤的参数进行比较。
术前24小时尿多巴胺在良性嗜铬细胞瘤中处于正常范围，但在恶性嗜铬细胞瘤中升高（P<0.0001）。香草扁桃酸在良性和恶性嗜铬细胞瘤中均升高，但恶性肿瘤中的水平高于良性肿瘤（P = 0.01）。尿肾上腺素和去甲肾上腺素样本检测未显示出差异。肿瘤位置分为肾上腺部位77例（81%）和肾上腺外部位18例（19%）。恶性嗜铬细胞瘤更常位于肾上腺外部位（P = 0.03）。家族性双侧嗜铬细胞瘤或多发性内分泌肿瘤患者中恶性肿瘤的发生率未增加。重量大于80 g的肿瘤与恶性肿瘤相关（P<0.0001）。恶性嗜铬细胞瘤中的多巴胺肿瘤浓度高于良性嗜铬细胞瘤（P = 0.01）。72例良性嗜铬细胞瘤中有9例（13%）出现持续性动脉高血压，10例恶性嗜铬细胞瘤中有6例（60%）出现持续性动脉高血压（P = 0.001）。良性嗜铬细胞瘤的10年生存率为94%。所有恶性嗜铬细胞瘤患者在此期间均死亡（P = 0.0001）。
术前24小时尿多巴胺水平高、肾上腺外肿瘤位置、肿瘤重量大、肿瘤多巴胺浓度升高以及术后持续性动脉高血压都是增加恶性嗜铬细胞瘤可能性的因素。具有这些特征的患者应更频繁地进行随访评估，以便在早期发现恶性肿瘤。
