散发性与家族性嗜铬细胞瘤临床特征及诊疗分析

目的:探讨嗜铬细胞瘤的临床特征，总结、提高嗜铬细胞瘤诊治水平。方法:对91例散发性嗜铬细胞瘤及5个家族中19例家族性嗜铬细胞瘤进行临床观察及30年随访。结果:散发性嗜铬细胞瘤患者91例，其中单侧多发性嗜铬细胞瘤2例，双侧嗜铬细胞瘤5例，肾上腺外嗜铬细胞瘤4例，单侧嗜铬细胞瘤79例，拟诊1例,经手术及病理证实90例，恶性2例，良性88例，手术后对侧复发1例,局部复发1例。5个家族的长期系谱随访调查中发现有19人发病，其中单侧嗜铬细胞瘤6例，双侧多发性嗜铬细胞瘤8例，其中并发甲状腺癌3例，拟诊5例,经手术及病理证实11例均为良性，术后对侧复发肿瘤3例。结论:散发性与家族性嗜铬细胞瘤在发病年龄、临床症状及相关生化检查、遗传学等方面仍具有一些差异，但在影像学及病理改变方面差别并不显著。

Clinical features and clinical analysis of sporadic and familial pheochromocytoma.

Objective : To evaluate the clinical features, diagnosis and treatment of pheochromocytoma . Methods: We carried out the clinical observations and 30-year follow-up visits in 91 cases of sporadic pheochromocytoma and 16 cases of familial pheochromocytoma in 5 kindreds. Results: In the 91 cases of sporadic pheochromocytoma, 1 with clinical suspection and 90 were confirmed by surgery and histopathoIogic examination (2 were malignant and 88 were benign) there were 2 cases of unilateral multiple pheochromocytoma,5 cases of bilateral pheochromocytoma, 4 cases of extra-adrenal pheochromocytoma,79 cases of unilateral pheochromocytoma. Among them, post-surgical contralateral recurrence was found in 1 and local recurrence was in 1 case too. The long-term follow-up survey in 5 kindreds revealed 19 cases of pheochromocytoma (6 unilateral, 8 bilateral multiple，with 3 cases being diagnosed as thyroid cancer), 5 cases were clinically suspected and 11 were confirmed by operation as benign tumors. Among the 11 surgical cases, contralateral tumor recurrence was found in 3 cases following operation. Conclusion: there are still some differences between sporadic and familial pheochromocytoma in terms of age of onset，clinical symptom, biochemical diagnosis and genetics. But It is difficult to distinguish between sporadic and familial pheochromocytoma in terms of imaging and pathological changes.