ECE2013 Poster Presentations Pituitary – Clinical (<emphasis role="italic">Generously supported by IPSEN</emphasis>) (127 abstracts)
Characteristics of patients with pituitary gigantism: results of an international study
Liliya Rostomyan 1 , Adrian F. Daly 1 , Maria Tichomirowa 1 , Luciana A. Naves 2 , Nalini Shah 3 , Philippe Chanson 4 , Sabina Zacharieva 5 , Constantine A Stratskis 6 , Sebastian Neggers 7 , Ian Holdaway 8 , Gunter K. Stalla 9 , Vyacheslav Pronin 10 , Dominique Maiter 11, , Jerome Bertherat 12 , Annamaria Colao 13 , Irena Ilovaiskaya 14 , Diego Ferone 15 , Margaret Zacharin 16 , Roberto Salvatori 17 & Albert Beckers 1
1Department of Endocrinology, CHU de Liège, Liege, Belgium; 2Department of Endocrinology, University of Brasilia, Brasilia, Brazil; 3Department of Endocrinology, Seth GS Medical College K.E.M. Hospital, Parel, Mumbai, India; 4Department of Endocrinology, CHU Le Kremlin-Bicetre, Le Kremlin Bicetre, France; 5Clinical Center of Endocrinology and Gerontology, Medical University, Sofia, Bulgaria; 6Section on Endocrinology Genetics, Program on Developmental Endocrinology Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), National Institute, Bethesda, Maryland, USA; 7Section of Endocrinology, Department of Internal Medicine, Erasmus Medical Centre, Rotterdam, The Netherlands; 8Department of Endocrinology, Greenlane Clinical Centre, Auckland, New Zealand; 9Department of Endocrinology, Max Planck Institute of Psychiatry, Munich, Germany; 10I.M. Sechenov First Moscow State Medical University, The Endocrinology Clinic, Moscow, Russia; 11Department of Endocrinology, St. Luc University Hospital, Université Catholique de Louvain, Brussels, Belgium; 12Department of Endocrinology, Institut Cochin, René Descartes University, Cochin Hospital, Institut National de la Santé et de la Recherche Médicale Unité 1016, Paris, France; 13Department of Molecular and Clinical Endocrinology and Oncology, “Federico II” University of Naples, Nalpes, Italy; 14Moscow Regional Research & Clinical Institute named by MF Vladimirsky, Moscow, Italy; 15Department of Endocrinology and Medical Sciences, Center of Excellence for Biomedical Research, University of Genoa, Genoa, Italy; 16Murdoch Childrens Research Institute, Royal Children’s Hospital and University of Melbourne, Parkville, Victoria, Australia; 17Division of Endocrinology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Aim: To analyse a large series of patients with pituitary gigantism.
Materials and methods: We included in this multicentre study 158 patients (129 males) with pituitary adenoma (PA) or hyperplasia and current/previous abnormal, excessively rapid growth velocity for age or a final height greater than 2 SD above normal for their population. Data of patients were systematically recorded in case report forms.
Results: The first symptoms developed at median age of 15 years (11–19). 96% had facial changes and/or acral overgrowth at time of diagnosis. Age at diagnosis: PA in females were younger than in males (16.5 vs 23 years) with median delay in diagnosis of PA of 5 years. Twenty-four patients were still growing and had median height of 191 cm (171–199). One hundred and thirty-four patients had stopped growing at age of 20 years (18–22). The relative difference from calculated midparental height was significantly greater in those with control of GH-excess after 20 years old than before (10.9% (8.4–15) vs 7.9% (5.9–10.3), P=0.012). Most PA were macroadenomas (84%), with median maximal tumor size of 25 mm (14.5–37). More than 50% of cases had extrasellar extension (77%) or invasion (54%). 4% had pituitary hyperplasia. One hundred and forty-five patients were operated with remission after first operation in 14% and in 0% in those who were reoperated (26 patients). Multimodal treatment approach was in 40% and disease control was achieved in 43% (median follow up on treatment was 7 years (2–16)). Hypopituitarism increased in frequency from 24% at baseline to 69% at last follow-up. Genetic/inherited features were seen in 34% at presentation and included syndromes like FIPA (with and without AIPmut), McCune-Albright, Carney complex, one case of familial pituitary hyperplasia. Overall, germline mutations in AIP gene were found in 43.6% (24/55) of those tested.
Conclusion: Patients with pituitary gigantism are predominantly male. Somatotropinomas in patients with gigantism are mostly large (with extrasellar extension and invasion in more than half of cases) and difficult to control. Treatment delay may increase the harm from GH-excess, particularly on tall stature. Syndromic features are presented in 1/3 of cases and AIP mutations are common in the tested population.
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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