ESPE Abstracts (2022) 95 P1-156

Hospital de Pediatria Garrahan, Buenos Aires, Argentina

Cushing’s disease (CD) is rare entity especially in pediatric patients. It is associated with significant morbidity. Early diagnosis and treatment is important for optimal outcome.

Aim: To evaluate the clinical, biochemical, pathology, treatments and outcome in a cohort of CD pediatrics patients in a single tertiary center.

Subjects and Methods: Retrospective review of 27 medical records of CD (chronological age (CA) <18 years (y) treated between 1992-2021. Data analyzed were: clinical, biochemical, radiological (MRI), bilateral inferior petrosal sinus sampling (BIPSS), histological features, therapeutic interventions and outcomes.

Results: CA at diagnosis (Mean ± SDS) 12.64 ± 2.52 years(y) (range(r) 5.88-17.32 y). F/M 1.45/1. Time between initial clinical signs and diagnosis was 1.44 ± 1.17 y. Features at diagnosis (%) were: Weight gain 92.5, Facial changes 77.8, striae 51.8, hirsutism 44.5, dorsal hump 44, acne 40.7, acanthosis nigricans 40.7, Hypertension 33.3, Pubertal delay or arrest 29.6, Growth retardation / Short stature 26, and others. Mean ± SD Height was -0.84 ± 1.09 (r:-2.68 to 0.03), weight 1.6± 1.46 (r: -0.54 to 4.76), BMI 1.78±0.76 (r: -0.7 to 2.8). Hypercortisolism was confirmed by UFC in 26/27 patients (96.2%) median 312 mg r/m2/day (r: 53-2005), SFC 23.00 hrs in 10/10 (100%) 1.06 ± 0.68 mg r/dl, Suppression test with dexametasone 18/18 (100%). High doses dexamethasone suppression test was positive in 15/16 patients, one patient did not inhibit. MRI showed microadenomas in 12 patients, macroadenomas in 7(3 invasive). Eight MRI (29,6 %) did not show adenoma. BIPSS were done in these patients. Central hypercortisolism were confirmed in 7 and one had ectopic Cushing (Carcinoide tumor in thymus). Transsphenoidal surgery (TSS) was performed in 22 /26 patients (excluding ectopic). 2 patients lost during follow-up, 1 received medical treatment and 1 craniotomy surgery. Histology confirmed ACTH+ adenoma in 17/23 (74%). The remission rates post-surgery was 66.6 % (16/24). Eight patients had persistent hypercortisolism. In these, second surgery, external pituitary Radiotherapy, gamma knife stereotactic radio-surgery, o medical treatments were underwent. Bilateral adrenalectomy was performed in one, who died. There was no recurrence after remission.

Conclusion: We reported the experience in our cohort of 27 CD patients seen in a single center over 30 years. The early diagnosis is essential to controlling hypercortisolism and prevents complications. Pituitary surgeon with experience of TSS in children is a priority. Post-surgical remission was similar to others series and same patients need second-line therapy. Recurrence is rare in this age group.

Volume 95

60th Annual ESPE (ESPE 2022)

Rome, Italy
15 Sep 2022 - 17 Sep 2022

European Society for Paediatric Endocrinology 

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