原发性肾上腺髓质增生症的诊断与治疗

①目的:探讨原发性肾上腺髓质增生症(PAMH)的诊断方法和治疗原则。②方法:回顾总结11例PAMH病人的临床资料并结合文献进行分析。③结果:影像学检查发现7例为一侧肾上腺增大、增厚，4例为双侧增生，均未发现肾上腺和肾上腺外肿瘤；11例病人均进行手术治疗并经病理学检查证实，术后效果良好。④结论:PAMH可靠的影像学诊断手段是CT和^131I-MIBG肾上腺髓质显像，确诊依赖于病理学检查特别是电镜下的发现；行增生明显的一侧全切、对侧大部切除术是有效的治疗方法。

DIAGNOSIS AND TREATMENT OF PRIMARY ADRENAL MEDULLAR HYPERPLASIA

Objective To investigate the diagnosis and treatment of primary adrenal medullar hyperplasia(PAMH). Methods The clinical data of 11 cases were reviewed and analyzed with related literatures. Results Imaging examination showed enlarged adrenal gland at one side in seven cases, and at both sides in the other four, no tumors were found. All patients underwent unilateral and/or bilateral adrenalectomy with good result. The diagnosis was confirmed histologically. Conclusion CT scan and 131 I-MIBG adrenal scintigraphy are objective and sensitive for the diagnosis of PAMH. The final diagnosis depends on pathology, especially on electron microscopy findings. The effective treatment is total removal of the herperplasic side, and sub-total of the opposite one.