Endocrine Abstracts

JOINT2127

We present the case of a 41-year-old woman with clinical symptoms of asthenia, proximal muscle weakness, behavioral changes, Cushingoid facies, visual disturbances and weight gain of 1.5 years. She also had a previous pelvic fracture with osteopenia in densitometry and hypertension of three months’ evolution. In the study due to clinical suspicion of Cushing’s syndrome, an elevation of urinary free cortisol was observed five times above the upper limit of normal, a dexamethasone suppression test of 1 mg of 26 mg/dl, elevated nocturnal salivary cortisol and inappropriately elevated ACTH repeated on three occasions. In ophthalmological study showed bilateral homonymous left quadrantanopia with bilateral left hemianoptic loss of sensitivity compatible with right parietotemporal lesion. Pituitary resonance imaging describes a giant pituitary mass measuring 5.1 × 3.5 × 4.2 cm with extensive growth to the interpeduncular cistern and prepontine cistern, extension through the floor of the sella turcica to the sphenoid sinus, invasion of the right cavernous sinus with hydrocephalus and signs of intracranial hypertension requiring placement of a shunt valve. Surgery was performed in 2022 first with transcranial surgery and required a second transsphenoidal surgery one month later due to significant tumor remnant. The main complications of the surgery were diabetes insipidus and hypothalamic syndrome with psychomotor agitation that was difficult to control, as well as secondary hypothyroidism and central catheter infection. The pathological study showed Ki67 of 4%, positivity for ACTH, alpha subunit, presence of Crooke cells and MGMT methylation and negativity for p53. In the hormonal study one month after surgery, the patient presented data of adrenal insufficiency with cortisol < 1 mg/dl and in the continuous resonance, the tumor residue was 3.7x2.7x2.2 cm. She received treatment with radiotherapy-protontherapy on the tumor residue, which remained stable in the following three years. Cushing’s disease due to invasive macroadenoma with positivity for Crooke cells occurs rarely. The positivity of these cells and MGMT methylation predispose to invasive macroadenomas with persistence of tumor residues and, in general, recurrence of Cushing’s disease. In our case, the patient remains without data of hypercortisolism and the tumor residue remains stable after three years of surgery and protontherapy.