目的:总结分析异位甲状旁腺腺瘤伴甲状旁腺功能亢进症的临床表现和诊治情况,以期提高对本病的认识。方法:收集2011年2月—2016年8月北京某医院收治的异位甲状旁腺腺瘤伴甲状旁腺功能亢进症7例,分析其临床资料和诊治情况并讨论相关的文献报道。结果:7例中,异位位于纵隔3例、胸腺3例、胸骨后1例,术后病理证实为甲状旁腺腺瘤6例,因鉴别困难倾向于甲状旁腺癌1例。确诊并给予手术切除腺瘤后,患者复查血钙及血清甲状旁腺素均恢复正常或较术前显著下降,骨痛等症状不同程度减轻。结论:异位甲状旁腺腺瘤合并甲状旁腺功能亢进较罕见、易误诊,了解甲状旁腺部位的多变性有助于异位甲状旁腺腺瘤及甲状旁腺功能亢进症的诊断和治疗。诊治关键在于术前明确定位,减少误诊误治,目前认为放射性核素检查准确率及阳性率更高,可为之后的治疗提供可靠依据。 Objective: To summarize and analyze the clinical manifestations and diagnosis and treatment of ectopic parathyroid adenoma with hyperparathyroidism, in order to raise awareness of the disease. Methods: Seven cases of ectopic parathyroid adenoma and hyperparathyroidism admitted from a hospital in Beijing from February 2011 to August 2016 were collected. The clinical data, diagnosis and treatment were analyzed and related literatures were discussed. Results: In 7 cases, 3 cases were located in the mediastinum, 3 cases in the thymus and 1 case in the sternum. The postoperative pathology was confirmed as parathyroid adenoma in 6 cases. Confirmed and given surgical resection of adenoma, the patient rechecked serum calcium and serum parathyroid hormone were returned to normal or significantly decreased compared with preoperative, such as bone pain and other symptoms to varying degrees. Conclusion: Ectopic parathyroid adenoma with hyperparathyroidism is rare and easily misdiagnosed. To understand the variability of the parathyroid gland is helpful for the diagnosis and treatment of ectopic parathyroid adenoma and hyperparathyroidism . The key lies in the diagnosis and treatment of a clear preoperative positioning to reduce misdiagnosis and mistreatment, the current rate of radionuclide examination and positive rate is higher, which can provide a reliable basis for the subsequent treatment.