Department of Neurosurgery, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China; School of Medicine, Tsinghua University, Beijing, China.
Department of Endocrinology, Chinese Academy of Medical Sciences and Peking Union Medical College, Peking Union Medical College Hospital, Beijing, China.
World Neurosurg. 2020 Oct;142:75-86. doi: 10.1016/j.wneu.2020.06.138. Epub 2020 Jun 24.
Ectopic adrenocorticotropic hormone-secreting pituitary adenomas (EAPAs) are rare adenomas causing Cushing syndrome, which are located in ectopic locations outside the sella turcica. No more than 100 cases of this entity have been reported. Because of its rarity and nonspecific clinical and biochemical features, EAPA is often misdiagnosed, leading to surgical failure and delayed treatment. In the article, the cases of 3 patients with suprasellar EAPAs treated at our institution are reported and a review of the literature is presented.
All 3 patients with Cushing syndrome had ectopic pituitary adenomas located in the suprasellar region and identifiable on preoperative sellar magnetic resonance imaging. Preoperative laboratory evaluation in all patients showed identical results to those observed in Cushing disease. In 2 patients, craniotomies were performed directly to achieve total tumor resection and clinical remission. One patient, who underwent previous negative transsphenoidal exploration, achieved full remission through a second craniotomy despite pulmonary infection as a postoperative complication.
Although EAPA is an extremely rare entity, it should be considered as a differential diagnosis of Cushing disease. Because of its similar clinical and biochemical behavior to intrasellar pituitary adrenocorticotropic hormone adenoma in Cushing disease, careful examination of the potential occurrence of EAPA on preoperative radiologic imaging is of considerable significance to avoid unnecessary surgery and achieve improved outcomes. Surgical resection EAPA remains the first choice of treatment, and the optimal surgical approach ought to be determined according to the adenoma features, the general condition of the patient, and the surgeon's experience.
异位促肾上腺皮质激素分泌垂体腺瘤（EAPA）是一种罕见的引起库欣综合征的腺瘤，位于鞍外的异位部位。这种实体瘤的病例报告不超过 100 例。由于其罕见性和非特异性的临床和生化特征，EAPA 常被误诊，导致手术失败和治疗延误。本文报告了我院治疗的 3 例鞍上 EAPA 患者的病例，并对文献进行了回顾。
所有 3 例库欣综合征患者的异位垂体腺瘤均位于鞍上区，术前鞍磁共振成像可识别。所有患者的术前实验室评估结果与库欣病观察到的结果相同。在 2 例患者中，直接进行开颅手术以实现肿瘤全切除和临床缓解。1 例患者曾行阴性经蝶窦探查术，尽管术后发生肺部感染等并发症，但通过第二次开颅手术达到完全缓解。
尽管 EAPA 是一种极为罕见的实体瘤，但应将其视为库欣病的鉴别诊断。由于其与库欣病中鞍内促肾上腺皮质激素腺瘤具有相似的临床和生化行为，因此在术前影像学检查中仔细检查 EAPA 的潜在发生具有重要意义，以避免不必要的手术并改善治疗效果。手术切除 EAPA 仍然是首选治疗方法，应根据腺瘤特征、患者一般状况和外科医生经验确定最佳手术方法。
