Endocrine Abstracts

Introduction: Despite their benign character, craniopharyngiomas tend to have an unpredictable evolution even after surgical treatment, while the onset of surgery-related growth hormone deficiency is frequently noted. As a part of the multihormonal process that promotes linear growth in GH-deficient children, other mechanisms (like hyperinsulinemia, hyperleptinemia, hyperprolactinemia, hypoestrogenism, low levels of IGF binding protein and possible GH variants) may be involved, aspects included under the concept of “growth without GH”.

Case presentation: We present a series of paediatric patients with craniopharyngioma regarding the progression of stature, weight and hormonal parameters. All underwent complete resection of the tumour and then developed GH deficiency (IGF-1 under -2 S.D). We also present the challenges faced during substitution therapy with recombinant GH (rGH). N.I. (14-year-old, female), M.D. (15-year-old, male) and D.D. (6-year-old, male) developed postoperative hyperphagia and hypothalamic obesity. During follow-up was noted a significant increase in stature (N.I currently at +3,03 S.D, M.D. at +2.65 S.D.), likely secondary to adiposity (latest BMI=38 kg/m², respectively 41,07 kg/m²), insulin resistance and hyperleptinemia. In the case of D.D., he reached +2,68 S.D. for weight, but his stature maintained in evolution a constant position on the growth curve at -1.49 S.D. Patient C.I. (12-year-old, female), initially referred for hypostature (-2.73 S.D), developed obesity after the tumour resection (latest BMI=29,1 kg/m², +2.56 S.D). Her stature progressed up to -0,73 S.D without replacement therapy.

Conclusion: Growth without GH is infrequent, often involving hyperinsulinemia through activation and redistribution of IGF-1. Independent of the alternative pathway involved, we observe that GH deficiency does not prevent linear growth in some children with craniopharyngioma. Substitution therapy during childhood with low dose rhGH is considered to be safe, given the pro-proliferative, angiogenic and anti-apoptotic effects of GH. It also positively impacts quality of life, body composition and fights against low bone mineral density and dyslipidemia.