Cushing’s Disease on Macroadenoma Developed in an Empty Sella Turcica: About a Case

Macroadenomas in cushing's disease represent 10% of cases, empty sella syndrome is relatively rare, it is defined as the extension of the subarachnoid space toward the intrasellar region with displacement of the pituitary to the postero-inferior wall. The prevalence of empty sella has been reported in up to 38% of imaging studies. Some patients may present with endocrine, neurological and ophthalmological symptoms due to the aberrant anatomy of the sellar region. The association with hormonal hypersecretion and in particular Cushing's syndrome is much rarer. We report the case of the B.Y patient aged 65 years without any previous history hospitalized in endocrinology for further exploration and management of a left lateral pituitary macroadenoma of 11 mm in height and 14 mm in wide in iso signal T1 and T2, pituitary gland laminated facing upwards, discovered following a hypertensive peak with neurological signs such as scintillating scotoma and dizzying sensations. The patient had some hallmark signs of Cushing's syndrome. Biological evaluation performed during the patient's first admission showed a broken circadian rhythm of cortisol with high midnight cortisol and basal ACTH level at 176 pg/mL. Urinary free cortisol was also elevated at 29.32 ug/dL. Two mg dexamethasone suppression test didn't show inhibition effect with cortisol level at 4.71 ug/dL. However, an inhibitory effect was observed with 8 mg dexamethasone test. A hyperprolactinemia of disconnection was associated with normal thyroid assessment (see Table 1). The associated comorbidities presented by our patient were diabetes mellitus, high blood pressure and osteopenia. The patient benefited from a transphenoidal adenoma resection with persistence of a high level of cortisol (D12: 23.56 ug/dL).Unfortunately, the patient is lost to follow-up. Conclusion: The singularity of our case remains in the association of two relatively rare circumstances associating a macroadenoma developed in an empty sella turcica both responsible for a hypercortisolism caused by an ACTH-secreting pituitary macroadenoma.