OBJECTIVE: The use of a growth hormone (GH) receptor antagonist, pegvisomant has shown great promise in adults with acromegaly, but experience in paediatric patients is lacking. We aimed to describe the results of pegvisomant therapy in a 12-year-old girl with an aggressive GH-secreting pituitary tumour. DESIGN: To evaluate the ability of pegvisomant therapy to control the effects of peripheral GH excess in a case of pituitary gigantism. METHODS: Pegvisomant was introduced at 10 mg/day, given subcutaneously, and gradually increased to 20 mg/day until serum IGF-I was normal for age. RESULTS: A large pituitary adenoma with suprasellar extension was diagnosed in a 12-year-old girl with progressive tall stature (178 cm), GH hypersecretion without suppression during oral glucose loading (nadir serum GH, 90 mU/l), high serum IGF-I and serum prolactin levels. Surgical extirpation was not possible because tumour tissue was fibrous and adherent to the optical nerves. Histological examination showed a mixed GH- and prolactin-secreting adenoma with lymphocytic infiltration of B and T cells. Treatment with a dopamine agonist, cabergoline, normalized serum prolactin, but GH secretion was resistant to both somatostatin analogue, octreotide and cabergoline. Radiation followed by pegvisomant therapy titrated up in dose to 20 mg/day led to a marked reduction in GH secretion and normalization of IGF-I, and to growth arrest and improvement of well-being. CONCLUSIONS: We suggest that treatment in pituitary gigantism with pegvisomant is safe and may normalize IGF-I levels and effectively stop growing.
OBJECTIVE: The use of a growth hormone (GH) receptor antagonist, pegvisomant has shown great promise in adults with acromegaly, but experience in paediatric patients is lacking. We aimed to describe the results of pegvisomant therapy in a 12-year-old girl with an aggressive GH-secreting pituitary tumour. DESIGN: To evaluate the ability of pegvisomant therapy to control the effects of peripheral GH excess in a case of pituitary gigantism. METHODS:Pegvisomant was introduced at 10 mg/day, given subcutaneously, and gradually increased to 20 mg/day until serum IGF-I was normal for age. RESULTS: A large pituitary adenoma with suprasellar extension was diagnosed in a 12-year-old girl with progressive tall stature (178 cm), GH hypersecretion without suppression during oral glucose loading (nadir serum GH, 90 mU/l), high serum IGF-I and serum prolactin levels. Surgical extirpation was not possible because tumour tissue was fibrous and adherent to the optical nerves. Histological examination showed a mixed GH- and prolactin-secreting adenoma with lymphocytic infiltration of B and T cells. Treatment with a dopamine agonist, cabergoline, normalized serum prolactin, but GH secretion was resistant to both somatostatin analogue, octreotide and cabergoline. Radiation followed by pegvisomant therapy titrated up in dose to 20 mg/day led to a marked reduction in GH secretion and normalization of IGF-I, and to growth arrest and improvement of well-being. CONCLUSIONS: We suggest that treatment in pituitary gigantism with pegvisomant is safe and may normalize IGF-I levels and effectively stop growing.
Authors: Liliya Rostomyan; Adrian F Daly; Patrick Petrossians; Emil Nachev; Anurag R Lila; Anne-Lise Lecoq; Beatriz Lecumberri; Giampaolo Trivellin; Roberto Salvatori; Andreas G Moraitis; Ian Holdaway; Dianne J Kranenburg-van Klaveren; Maria Chiara Zatelli; Nuria Palacios; Cecile Nozieres; Margaret Zacharin; Tapani Ebeling; Marja Ojaniemi; Liudmila Rozhinskaya; Elisa Verrua; Marie-Lise Jaffrain-Rea; Silvia Filipponi; Daria Gusakova; Vyacheslav Pronin; Jerome Bertherat; Zhanna Belaya; Irena Ilovayskaya; Mona Sahnoun-Fathallah; Caroline Sievers; Gunter K Stalla; Emilie Castermans; Jean-Hubert Caberg; Ekaterina Sorkina; Renata Simona Auriemma; Sachin Mittal; Maria Kareva; Philippe A Lysy; Philippe Emy; Ernesto De Menis; Catherine S Choong; Giovanna Mantovani; Vincent Bours; Wouter De Herder; Thierry Brue; Anne Barlier; Sebastian J C M M Neggers; Sabina Zacharieva; Philippe Chanson; Nalini Samir Shah; Constantine A Stratakis; Luciana A Naves; Albert Beckers Journal: Endocr Relat Cancer Date: 2015-07-17 Impact factor: 5.678
Authors: Albert Beckers; Maya Beth Lodish; Giampaolo Trivellin; Liliya Rostomyan; Misu Lee; Fabio R Faucz; Bo Yuan; Catherine S Choong; Jean-Hubert Caberg; Elisa Verrua; Luciana Ansaneli Naves; Tim D Cheetham; Jacques Young; Philippe A Lysy; Patrick Petrossians; Andrew Cotterill; Nalini Samir Shah; Daniel Metzger; Emilie Castermans; Maria Rosaria Ambrosio; Chiara Villa; Natalia Strebkova; Nadia Mazerkina; Stéphan Gaillard; Gustavo Barcelos Barra; Luis Augusto Casulari; Sebastian J Neggers; Roberto Salvatori; Marie-Lise Jaffrain-Rea; Margaret Zacharin; Beatriz Lecumberri Santamaria; Sabina Zacharieva; Ee Mun Lim; Giovanna Mantovani; Maria Chaira Zatelli; Michael T Collins; Jean-François Bonneville; Martha Quezado; Prashant Chittiboina; Edward H Oldfield; Vincent Bours; Pengfei Liu; Wouter W de Herder; Natalia Pellegata; James R Lupski; Adrian F Daly; Constantine A Stratakis Journal: Endocr Relat Cancer Date: 2015-02-24 Impact factor: 5.678
Authors: A Giustina; A Barkan; P Chanson; A Grossman; A Hoffman; E Ghigo; F Casanueva; A Colao; S Lamberts; M Sheppard; S Melmed Journal: J Endocrinol Invest Date: 2008-09 Impact factor: 4.256