Koutourousiou Maria, Winstead Welby I
Department of Neurological Surgery, University of Louisville, Louisville, Kentucky, USA.
Department of Otolaryngology, University of Louisville, Louisville, Kentucky, USA.
World Neurosurg. 2017 Feb;98:870.e5-870.e10. doi: 10.1016/j.wneu.2016.12.021. Epub 2016 Dec 18.
Complete surgical resection of an adrenocorticotropic hormone (ACTH)-secreting pituitary adenoma is the gold standard of treatment of Cushing disease. Ectopic location of these adenomas is an extremely rare condition that may compromise the diagnosis and surgical success. We present the first case of an ectopic intracavernous ACTH-secreting macroadenoma totally resected with endoscopic endonasal surgery (EES).
A 36-year-old woman presented with Cushing syndrome. Increased ACTH, serum cortisol, and free urine cortisol levels were identified; however, pituitary magnetic resonance imaging failed to show a pituitary tumor; instead, a parasellar lesion in the left cavernous sinus (CS) was noticed. Inferior petrosal sinus sampling showed a significant central to peripheral and lateralized left-sided ACTH gradient. The patient underwent EES. No tumor was found in the sella; however, the left CS was widely explored and a tumor was found lateral to the paraclival segment of the carotid artery. There were no complications after EES. Pathology confirmed the diagnosis of an ACTH-secreting adenoma. During the immediate postoperative course, serum cortisol levels decreased lower than 5 μg/dL. Postoperative magnetic resonance imaging showed complete tumor resection. At 20 months follow-up, the patient remained in clinical and biochemical remission of Cushing disease.
Only 12 cases of ectopic intracavernous ACTH-secreting adenomas have been reported and all were microadenomas. The presence of an ectopic ACTH-secreting macroadenoma in the CS represents a surgical challenge. EES is the ideal approach for complete resection of ectopic intracavernous adenomas, allowing for a wide exploration of the CS with no surgical complications.
促肾上腺皮质激素（ACTH）分泌型垂体腺瘤的完整手术切除是库欣病治疗的金标准。这些腺瘤的异位定位是一种极其罕见的情况，可能会影响诊断和手术成功率。我们报告首例经鼻内镜手术（EES）完全切除的海绵窦内异位ACTH分泌型大腺瘤病例。
一名36岁女性出现库欣综合征。检测到促肾上腺皮质激素、血清皮质醇和游离尿皮质醇水平升高；然而，垂体磁共振成像未显示垂体肿瘤；相反，发现左侧海绵窦（CS）有一个鞍旁病变。岩下窦采血显示中央到外周有显著的促肾上腺皮质激素梯度，且左侧更为明显。患者接受了EES。蝶鞍内未发现肿瘤；然而，对左侧海绵窦进行了广泛探查，在颈内动脉岩骨段旁发现了一个肿瘤。EES术后无并发症。病理证实为ACTH分泌型腺瘤。术后即刻，血清皮质醇水平降至低于5μg/dL。术后磁共振成像显示肿瘤完全切除。随访20个月时，患者的库欣病在临床和生化方面均处于缓解状态。
仅报道了12例海绵窦内异位ACTH分泌型腺瘤病例，且均为微腺瘤。海绵窦内存在异位ACTH分泌型大腺瘤是一项手术挑战。EES是完全切除海绵窦内异位腺瘤的理想方法，可广泛探查海绵窦且无手术并发症。
