Endocrine Abstracts

The spontaneous remission of Cushing disease (CD) might be a phase of cyclic disease, but could also be explained by an ACTH-microadenoma infarction or hemorrhage, with clinical pictures ranging from asymptomatic hypercortisolism normalization to dramatic hypocortisolism. We describe the case of a 23-years-old woman, referred for amenorrhea lasting for 5 months, overweight and acne. Physical examination showed mild hirsutism, acne, round face with a hint at rubeosis, abdominal striae rubrae, normal BP. Biochemical examination showed IGT; lipids, kalemia, thyroid function, PRL and androgens were normal. Hypercortisolism was confirmed by elevated UFC (143-361 μg/24 h, nv 20–90), supranormal h24-cortisol(F), lack of cortisol suppression after LDDT. Cortisol suppressed normally after HDDT, ACTH increased by 36% after hCRH, ACTH and cortisol increased by 350 and 80% after DDAVP-test. Pituitary MRI was not conclusive for a microadenoma. BIPSS, performed 40 days later, did not show a C/P-ACTH gradient, but UFC value in the day before the procedure turned out to be normal. In the following months UFC was normal and menses resumed in spite of persistent cushingoid features. Two months later the patient was hospitalized with severe headache, fatigue, diffuse unspecific pain. Unequivocal hypocortisolism was found (morning F 0,1 μg/dl, no response to ACTH-1μg). Symptoms remitted under substitutive therapy, DDAVP-test turned out to be negative. MRI showed an hypointense area smaller than that previously seen, suggesting a progressive disappearance of a microadenoma. After five months all cushingoid features had disappeared, weight had decreased by 7 kg, menses regularized. After six months the patient stopped substitutive therapy, with a normal response to ACTH-1μg two months later; MRI after one year was normal. We concluded for spontaneous apoplexy in ACTH-microadenoma, not related to BIPSS or dynamic tests. Nowadays we cannot still exclude a cyclic CD: a prolonged follow up is mandatory to discern between these possible conditions.