Successful treatment of Cushing’s disease caused by ectopic intracavernous microadenoma

Abstract

Adrenocorticotropic hormone (ACTH)-secreting pituitary adenomas are sometimes difficult to visualize, even with high-quality magnetic resonance imaging, due to their small size and variable location. Sampling the cavernous or inferior petrosal sinus is helpful for confirming the central origin of a tumor, but ectopic corticotroph adenomas in the paraseller region also typically exhibit a high central/peripheral plasma ACTH ratio. We experienced an extremely rare case of Cushing's disease caused by an ACTH-secreting microadenoma located entirely inside the left cavernous sinus attached to the medial wall (ectopic pituitary adenoma) that was not visible by preoperative MRI. In this case, the microadenoma was completely removed and an endocrinologic cure was achieved. This case reveals that in addition to meticulous sectioning of the pituitary gland, bilateral periglandular inspection with visualization of the medial wall of the cavernous sinus and of the diaphragm should always be performed to detect ectopic parasellar microadenomas when no adenoma is visible by preoperative MRI.

Therapeutic outcomes in patients undergoing surgery after diagnosis of Cushing’s disease: A single-center study

“…Of these 22 patients, three died of pneumonia, systemic fungal infection, or renal failure within one year after surgery. One patient underwent surgery four times suggesting an ectopic pituitary adenoma [6]. In contrast, there was no postoperative improvement in disease in the remaining seven patients with no adenomas during surgery.…”

“…Of these 252 patients, tumors were not visible on extensive preoperative MRI studies (including 3T MRI) in 22 patients (8.7%), whereas microadenoma or macroadenoma was suspected in the remaining 230 patients based on MRI (154 microadenoma patients and 76 macroadenoma patients). These 230 cases included two ectopic microadenomas located within the cavernous sinus (CS) [6], three double adenomas associated with nonfunctioning macroadenoma, GH and PRL microadenoma, and PRL microadenoma [7], two invasive macroadenomas that progressed to pituitary carcinoma associated with liver metastases or dissemination to the central nervous system [8]. However, there were no tumors with Nelson syndrome in this series.…”

A pitfall in diagnosing Cushing’s disease: ectopic ACTH-producing pituitary adenoma in the sphenoid sinus

“…in the cavernous sinus. A rare cause is an ectopic pituitary adenoma, a few of which have been described within the cavernous sinus [3,4,11,13] or elsewhere [5][6][7][8]. Here, we report a rare case of an ectopic pituitary adenoma within the sphenoid sinus.…”

“…Ectopic pituitary adenomas are extremely rare [4,8]. These tumors mostly arise in the proximity of the pituitary gland, e.g.…”

Ectopic ACTH-secreting pituitary adenoma of the sphenoid sinus: case report of endoscopic endonasal resection and systematic review of the literature

“…14,31 Given the high mortality rate for those with nonpituitary Cushing syndrome (44% at 10 years, in stark contrast to just 3% with Cushing disease), 30 the prompt identification and removal of a functioning ectopic pituitary adenoma is essential and can result in a rapid and durable remission. 18,21 Given the severity of the disease, patients presenting with Cushing syndrome from an unknown source should always be evaluated for the possibility of an ectopic pituitary adenoma. These tumors are exceedingly rare; roughly 90 cases of all types of ectopic pituitary adenomas 17 have been reported since Erdheim published the first case in 1909.…”