Background: Pituitary gigantism is an extremely rare disorder characterised by GH excess that occurs before fusion of the epiphyseal growth plates.
Case summary: A 13 years 6 month old Chinese boy presented to the paediatric endocrine outpatient clinic with tall stature. He was noted by his parents to have a continued growth spurt since 9 years old, and he required new shoes and clothes every school term. He had no headaches or visual disturbances. There is no family history of tall stature. On examination, his height measured 191.3 cm (> 2 SD above the 97th percentile) and his weight measured 66.0 kg (90th-97th percentile). He had no gynaecomastia or features of Marfan syndrome. There was mild prognathism. He was Tanner Stage 4 for pubic hair and his testicular volumes measured 15 ml bilaterally. His bone age was 14 years with a predicted height of 206.0 cm. An oral glucose suppression test (OGTT) showed a failure of GH suppression with a paradoxical rise after 60 min to a peak GH of 25.0 ug/L and an elevated IGF1 of 877.0 ug/L (183850). Serum prolactin was normal at 198 mIU/L (72.0320.0). A pituitary MRI showed a pituitary macroadenoma measuring 12×9×7 mm. Perimetry was normal. He underwent endonasal transphenoidal hypophesectomy and he developed central diabetes insipidus post operatively. Histology confirmed a pituitary adenoma which was positive for growth hormone and prolactin. A repeat OGTT 3 months after surgery showed adequate suppression of GH levels of 0.73 ug/L with normalisation of IGF1 to 228 ug/L (183850) and a pituitary MRI showed no tumour recurrence.
Conclusion: We report a case of GH excess secondary to a pituitary macrodenoma which achieved surgical and biochemical cure with transphenoidal surgery.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology