Women affected by Cushings disease (CD) are often infertile due to abnormal follicular development or anovulation. In these patients, first line treatment is pituitary surgery, which is effective in more than 83% of cases with microadenomas, but only in about 35% of those with macroadenomas. When surgery do not normalize circulating ACTH and cortisol concentrations, such drugs, e.g. ketoconazole or metirapone, and radiotherapy are used as adjuvant treatment. Concerning radiotherapy, more precise and focused approaches of delivering larger amounts of radiation have been introduced recently in the management of pituitary tumors. Cyber-knife radiosurgery (CKR) is considered as a possible treatment for patients affected by unsuccessfully surgically treated pituitary adenoma or not suitable for surgery. The disadvantages of this technique seem to be the length of time to the onset of remission, which is known to be at least of 6 months, and the possible adverse effects. In patients treated with radiotherapy, however, mild hypercortisolism can persist for a long time and fertility could not be restored, even when menses have been normalized. We report here a case of a 37-yr-old female patient with Cushings disease (CD) due to ACTH-secreting 5 mm adenoma on the left side of the pituitary. Because complete clinical remission was not obtained by a transsphenoidal surgery in 2010, CKR was performed for to achieve remission in January 2011. Thereafter, treatment with ketoconazole, at the dose of 400 mg daily, normalized serum cortisol and UFC levels, allowing the recovery of regular menses. Because of low FT4 and low TSH and undetectable AbTPO values, L-T4 replacement was started. 5 years after CKR, morning ACTH, cortisol concentrations, 24-h urinary free cortisol (UFC) and cortisol levels after 1-mg dexamethasone (1-mg dex) administration were normalized. MRI also showed partial shrinkage of the pituitary tumor. The patient, who had regular menses, became pregnant 60 months after CKR. During pregnancy, plasma ACTH, serum cortisol and UFC levels increased. Fasting glucose levels (FGL), HbA1c and blood pressure were normal all throughout pregnancy. Overall, patients body weight increase was 13.6 kg. At the 38th week of an uneventful pregnancy, the patient delivered vaginally a healthy female newborn (length 48 cm, weight 2600 g). The baby and the mother did not show any biochemical signs or clinical symptoms of hypo- or hypercortisolism. In conclusion, pregnancy should be considered at risk in patients with CD, even the patient has normalized cortisol levels. Moreover, periodic evaluation of pituitary function is mandatory during gestation, due to the high risk of hypopituitarism.
20 May 2017 - 23 May 2017