Endocrine Abstracts

Introduction: Gigantism represents a rare pathology in children, characterized by growth hormone (GH) excess, before epiphyseal fusion. It associates significant morbidity and decreased life expectancy, in lack of appropriate treatment.

Case presentation: O.D., a male patient, 16 years old now, has been followed in our clinic since 2016, being diagnosed with gigantism in Bega Pediatric Clinic, at the age of 12 years 9 months. In 2014, the patient presented visual disturbances, headache, soft tissue swelling, accelerated growth in height (weight 85 kg, height 171 cm, S.D. = + 2.6). Laboratory data confirmed high values of IGF-1 and basal GH, respectively non-suppressive GH at oral glucose tolerance test. No other pituitary insufficiency was noted. Pituitary MRI revealed a 3.9 cm pituitary macroadenoma. Transsphenoidal adenomectomy was performed, but only a part of the tumor could be removed (tumor remnant volume: 34/20/30 mm). Postoperatively, somatostatin analogue treatment was started. Later, due to the incomplete response (with persistent sleep apnea and headache), dopamine agonists (cabergoline) were added. In 2016 the patient was operated again, without complete removal of the tumor. In addition, partial pituitary insufficiency (on gonadotrophs and thyrotrophs) developed. Pituitary MRI shows a tumor remnant of 1.9 cm. Pegvisomant was added to somatostatin analogues and cabergoline treatment. In 2017, due to the persistence of symptomatology and the unsatisfactory response to the medical treatment, gamma-knife radiosurgery was performed. Also, the somatostatin analogue was changed. Up to now, the disease is well controlled under treatment.

Conclusion: Advances in the pharmacotherapy and radiosurgery in patients with acromegaly and gigantism allow successful control of the disease. Precocious diagnosis is important, many of the complications being reversible in initial stages.