+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Endoscopic Endonasal Surgery for Remission of Cushing Disease Caused by Ectopic Intracavernous Macroadenoma: Case Report and Literature Review



Endoscopic Endonasal Surgery for Remission of Cushing Disease Caused by Ectopic Intracavernous Macroadenoma: Case Report and Literature Review



World Neurosurgery 98: 870.E5-870.E10



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.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 059680487

Download citation: RISBibTeXText

PMID: 28003168

DOI: 10.1016/j.wneu.2016.12.021


Related references

Cushing Disease Caused By an Ectopic Intracavernous Pituitary Microadenoma: Case Report and Review of the Literature. Journal of Computer Assisted Tomography 27(3): 424-426, 2003

Case report: cushing disease caused by an ectopic intracavernous pituitary microadenoma: case report and review of the literature. Journal of Computer Assisted Tomography 27(3): 424-426, 2003

Ectopic intracavernous sinus adrenocorticotropic hormone-secreting microadenoma: could this be a common cause of failed transsphenoidal surgery in Cushing disease? Case report. Journal of Neurosurgery 98(6): 1312-1317, 2003

Ectopic ACTH-secreting pituitary adenoma of the sphenoid sinus: case report of endoscopic endonasal resection and systematic review of the literature. Neurosurgical Focus 38(2): E10, 2015

Spontaneous remission of Cushing's disease. A case report and review of the literature. American Journal of Medicine 67(3): 519-523, 1979

Navigation-assisted endoscopic endonasal surgery of a glomangiopericytoma with intraorbital extension: A case report and literature review. Ci Ji Yi Xue Za Zhi 30(2): 119-121, 2018

Pituitary macroadenoma and Cushing's disease in pediatric patients: patient report and review of the literature. Journal of Pediatric Endocrinology and Metabolism 11(5): 665-669, 1998

Successful treatment of Cushing's disease caused by ectopic intracavernous microadenoma. Pituitary 14(3): 295-298, 2011

Invasive ACTH-secreting pituitary macroadenoma in remission after transsphenoidal resection: A case report and literature review. Medicine 97(46): E13148, 2018

Evaluation of Outcomes After Endoscopic Endonasal Surgery for Large and Giant Pituitary Macroadenoma: A Retrospective Review of 39 Consecutive Patients. World Neurosurgery 84(4): 978-988, 2015

Management of postoperative vasospasm following endoscopic endonasal surgery for craniopharyngioma: Report and review of literature. Neurology India 67(2): 606-609, 2019

Endoscopic endonasal resection of the odontoid peg--case report and literature review. Child's Nervous System 28(10): 1795-1799, 2012

Endoscopic endonasal resection of clival xanthoma: case report and literature review. Neurosurgical Review 38(4): 765-769, 2015

Intracavernous sinus ectopic adrenocorticotropin-secreting tumours causing therapeutic failure in transsphenoidal surgery for Cushing's disease. Acta Neurochirurgica 142(8): 855-864, 2000

Ectopic Cushing syndrome in small cell lung cancer: A case report and literature review. Thoracic Cancer 8(2): 114-117, 2017