ESPE Abstracts

Patient 10 years old, female, since 8 years old of excessive weight gain, facial changes, hirsutism, violaceous striae on trunk, abdomen and legs, acanthosis nigricans, dorsal cervical fat pad, muscle weakness and sleep disturbances. Menarche at 9 years old. Weight 110.4 kg, height 141.6 cm (+0.08 SD), BMI 55.1 (+4.67 SD). Initial study HBA1C 5.17%, glycemia 86 mg/dl DHEAS110 ug/dl, Total testosterone 39.19 ng/dl, SHBG10nmol/l, FAI 3.09, Androstenedione 2.5 ng/ml, 17 hydroxiprgesterone 1.04 ng/ml, urinary free cortisol 937.5 ug/24 hours adjusted by creatinuria 806ug/gr. Cortisol post 1 mg dexamethasone 48.88 ug/dl, ACTH 45.2 pg/ml, nocturnal salivary cortisol sample 1: 0.4 ug/dl Sample 2: 0.26 ug/dl (VN <0.1). Initial pituitary resonance imaging showed no lesions. She was referred to a more complex center for study due to suspicion of Cushing's disease. A new pituitary MRI was performed in a specialized center which shows a pituitary gland with a convex upper edge. It presents a heterogeneous reinforcement structure after the administration of gadolinium. Hypoenhancing pseudonodular area occupies the anterior aspect of the sellar lodge, measuring approximately 5 × 5 × 15mm in the long axes. Neuro ophthalmological evaluation without injuries. Pre-surgery examinations: Cortisol am 138 ng/ml Cortisol pm 158 ng/ml, ACTH 42.7pg/ml GH 0.04 ng/ml Prolactin 11.8 ng/ml TSH 0.66 uIU/ml FT4 0.99 ng/dl LH 1.7 mIU/ml FSH <0.1 mIU/ml estradiol 51 pg/ml Total testosterone 0.54 ng/ml. Pituitary tumor excision was performed through transsphenoidal access, samples were sent for genetic study, results pending to date. Post surgery examinations: ACTH 18.6 pg/ml TSH 2.91 T4 L 1.8 post cortisol 17 ng/ml.

Discussion: Cushing's disease is rare in pediatrics, its diagnosis is a challenge; In the case of our patient, 2 years passed from the first symptoms to her diagnosis; It is important that despite being infrequent, do not forget its presentation and always evaluate with a team with multidisciplinary experience.