以嗜铬细胞瘤为主要表现的多发性内分泌腺瘤

目的：探讨以嗜铬细胞瘤为主要表现的多发性内分泌腺瘤（MEN）的诊治。方法：3例以嗜铬细胞瘤为主要表现的MEN，均行单侧肾上腺切除术，其中例1 、例2为同胞组妹均诊断为嗜铬细胞瘤并发甲状腺髓样癌，属MRN－Ⅱa型，行甲状腺全切加同侧淋巴结清扫术；例3为嗜铬细胞瘤并发胰岛素瘤，属MEN混合型，同时行嗜铬细胞瘤、胰岛素瘤切除术。结果：3例手术切除效果良好。例1术后6年死于其他疾病，例2术后已生存23年无复发，例3术后血压、血糖、血胰岛素恢复正常，随访6个月无复发。结论：提高对本病的认识，选择适当的内分泌和影像学等检查手段，是早期诊断的关键。MEN中嗜铬细胞并发其他肿;且先行嗜铬细胞瘤切除术，是否全切双侧肾上腺应依具有体情况而定，然后再处理其他肿瘤。

Multiple endocrine neoplasia with main manifestation of pheochromocytoma

Purpose:To explore the methods of diagnosis and treatment formultiple endocrine neoplasia with main manifestation of pheoch romocytoma. Methods:Three cases were reported. Two sisters of the three cases were diagnosed as pheochromocytoma and medullary thyroid carcino ma (MEN-Ⅱa) . They were treated by adrenalectomy and thyroidectomy.The third p atient who was diagnosed as pheochromocytoma and insulinoma was removed by adren alectomy and insulinomaectomy at the same time. Results:Three patients were cured by operation. One patien t has been followed up for 23 years without recurrence, the other died of esopha gus six years later without recurrence of MEN. The third patient has kept normal blood pressure and glucose for six months.Conclusions:Clinical findings of multiple endocrine neopla sia are variable in different cases. The necessary checks of endocrine and imag ing are very important in earlier diagnosis. It is effective to remove the tum ours by surgery.