恶性嗜铬细胞瘤(附12例报告)

Malignant pheochromocytoma (report of 12 cases)

目的 提高恶性嗜铬细胞瘤诊治水平。 方法 总结 12例恶性嗜铬细胞瘤患者临床资料。 结果 12例均行手术治疗 ,1例术后 2 4h死于顽固性低血压 ,余 11例随诊 2年 6个月～ 13年 ,平均 5年 6个月。首次手术根据肿瘤浸润及局部淋巴结转移情况确诊恶性嗜铬细胞瘤 5例中 ,1例膀胱恶性嗜铬细胞瘤行膀胱部分切除术后 ,出现顽固性低血压 ,抢救无效于术后 2 4h死亡 ;余 4例存活 2～ 3年死于肿瘤复发及高血压并发症。 7例首次手术病理诊断为嗜铬细胞瘤者 ,术后 10个月～ 5年肿瘤复发 ,病程中发现肿瘤转移至肝 3例 ,肝、肺、骨均有转移者 2例 ,局部淋巴结转移 2例。存活时间 <2年者 3例 ,3～ 11年者 4例 (包括目前存活 3例 )。 结论 病理难以区别肿瘤的良恶性 ,现代影像学检查 (CT、MRI等 )可为恶性嗜铬细胞瘤的诊断提供参考依据。对直径 >5cm ,内部结构不均匀的复发性嗜铬细胞瘤 。

Objective To evaluate the diagnosis and treatment procedures for malignant pheochromocytoma. Methods 12 cases of malignant pheochromocytoma surgically treated were reviewed.This series comprised of 4 male and 8 female patients.All the tumors were confirmed on surgery and pathological studies. Results All the cases were operated.One died of hypotension after surgery.11 cases have been followed up for 2.5～13.0 years.4 died of recurrence and complication of hypertension.3 died of extensive metastases more then one year after operation.One died of pulmonary infection 4 years after operation and the others have been surviving. Conclusions Imaging procedures are important for the diagnosis of malignant pheochromocytoma.Recurrent tumors greater than 5 cm in diameter and uneven density in the tumor denote malignancy.Prompt radical extirpation of the tumor is the only means to achieve a long survival.