不同临床表现类型的嗜铬细胞瘤

Clinical types of pheochromocytom

目的 提高对嗜铬细胞瘤的诊断和治疗水平。 方法 总结 1987年 6月～ 1998年 8月诊治的嗜铬细胞瘤 90例。 结果 本组 90例中 3 6例 5 1例次表现为 :肾上腺外者 14例 ,无症状性5例 ,复发性 8例 ,恶性或恶变者 15例 ,双侧或多发性 4例 ,家族性及多发性内分泌腺瘤 5例。 3 6例嗜铬细胞瘤随访 6个月～ 10年 ,8例复发 ,恶变 3例。 结论 手术切除肿瘤是嗜铬细胞瘤有效的治疗方法。复发性嗜铬细胞瘤并不都是恶性肿瘤 ,但因恶性倾向较大 ,需密切随诊。家族性嗜铬细胞瘤常为双侧或多发性。

Objective To improve the diagnosis and treatment of pheochromocytoma. Methods 90 patients with pheochromocytomas were treated from June 1987 to August 1998. These patients showed extra adrenal (14 patients), non symptomatic (5), recurrent (8), malignant (15), bilateral or multiple (4), and familial or MEN IIA (5). Results One patient with malignant extra adrenal pheochromocytoma showed extensive metastasis. All the patients except this patient were confirmed pathologically. 36 patients were followed up for 5 months～10 years. Eight patients had recurrence and 3 showed malignancy. Conclusions Open surgery is an effective method for pheochromocytoma. Preoperative preparation must be sufficient to mabe the operation safe. Recurrent pheochromocytomas are not malignant in nature, but they have considerable malignant tendency and should undergo intensive surveillance and regular examinations. Familial pheochromocytomas are bilateral or multiple. They could develop into secondary medullar thyroid carcinoma in the meantime.