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1. Erratum to: Prospective, long-term study of the effect of cabergoline on valvular status in patients with prolactinoma and idiopathic hyperprolactinemia

Author

Albert Beckers, Mathilde Rubio-Almanza, Ibrahima Maiga, Julien Magne, Adrian Daly, Raluca Elena Dulgheru, Monica Tome Garcia, Renata S. Auriemma, Laurent Vroonen, Patrizio Lancellotti, Patrick Petrossians, CHU de Liège (Endocrinologie), service d'endocrinologie, Service de cardiologie [Liège], CHU de Liège-Domaine Universitaire du Sart Tilman, Neuroépidémiologie Tropicale (NET), CHU Limoges-Institut d'Epidémiologie Neurologique et de Neurologie Tropicale-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST), Université de Limoges (UNILIM)-Université de Limoges (UNILIM), Service de cardiologie [CHU Limoges], CHU Limoges, Service d'Endocrinologie (LIEGE - Endocrino), and Université de Liège

Subjects
medicine.medical_specialty, Pediatrics, business.industry, Endocrinology, Diabetes and Metabolism, 030209 endocrinology & metabolism, Retrospective cohort study, Pituitary neoplasm, medicine.disease, 3. Good health, Surgery, 03 medical and health sciences, 0302 clinical medicine, Endocrinology, Interquartile range, 030220 oncology & carcinogenesis, Diabetes mellitus, Cabergoline, medicine, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Young adult, Prospective cohort study, business, Prolactinoma, ComputingMilieux_MISCELLANEOUS, medicine.drug
Abstract

Since the 1990's cabergoline has been the treatment of choice in prolactinoma, as it permits rapid and effective hormonal and tumor control in most cases. Evidence of cardiac valvulopathy was demonstrated in Parkinson's disease patients treated with dopamine agonists. Retrospective studies in prolactinoma patients treated with cabergoline at lower doses did not show such an effect. However, few prospective data with long-term follow-up are available. The aim of this study was to assess the safety of cabergoline regarding cardiac valvular status during prospective follow-up in patients treated for prolactinoma or idiopathic hyperprolactinemia. We report here a series of 100 patients (71F; median age at diagnosis: 41.5 years) treated with cabergoline for endocrine diseases (prolactinoma n = 89, idiopathic hyperprolactinemia n = 11). All patients underwent complete transthoracic echocardiographic studies at baseline and during long-term prospective surveillance using the same equipment and performed by the same technicians. The median interval between baseline and last follow-up echocardiographic studies while on cabergoline was 62.5 months (interquartile range: 34.75-77.0). The median total duration of cabergoline treatment was 124.5 months (interquartile range: 80.75-188.75) and the median cumulative total dose of cabergoline was 277.8 mg (interquartile range : 121.4-437.8 mg) at last follow-up. We found no clinically relevant alterations in cardiac valve function or valvular calcifications with cabergoline treatment. Our data suggest that findings from retrospective analyses are correct and that cabergoline is a safe chronic treatment at the doses used typically in endocrinology.

Published
2017
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2. T2-weighted MRI signal predicts hormone and tumor responses to somatostatin analogs in acromegaly

Author

Iulia Potorac, Patrick Petrossians, Adrian F Daly, Orsalia Alexopoulou, Sophie Borot, Mona Sahnoun-Fathallah, Frederic Castinetti, France Devuyst, Marie-Lise Jaffrain-Rea, Claire Briet, Florina Luca, Marion Lapoirie, Flavius Zoicas, Isabelle Simoneau, Alpha M Diallo, Ammar Muhammad, Fahrettin Kelestimur, Elena Nazzari, Rogelio Garcia Centeno, Susan M Webb, Marie-Laure Nunes, Vaclav Hana, Véronique Pascal-Vigneron, Irena Ilovayskaya, Farida Nasybullina, Samia Achir, Diego Ferone, Sebastian J C M M Neggers, Brigitte Delemer, Jean-Michel Petit, Christof Schöfl, Gerald Raverot, Bernard Goichot, Patrice Rodien, Bernard Corvilain, Thierry Brue, Franck Schillo, Luaba Tshibanda, Dominique Maiter, Jean-François Bonneville, Albert Beckers, Centre Hospitalier Universitaire de Liège (CHU-Liège), Université Catholique de Louvain = Catholic University of Louvain (UCL), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Assistance Publique - Hôpitaux de Marseille (APHM), Université libre de Bruxelles (ULB), Università degli Studi dell'Aquila () (Univaq), Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Centre Hospitalier Universitaire de Strasbourg (CHU de Strasbourg ), Centre Hospitalier Universitaire de Lyon, University Hospital Erlangen, Unité de Génétique Moléculaire Animale (UMR GMA), Université de Limoges (UNILIM)-Institut National de la Recherche Agronomique (INRA), Centre Hospitalier Universitaire de Reims (CHU Reims), Erasmus University Rotterdam, Erciyes University, University of Genoa (UNIGE), Hospital General Universitario 'Gregorio Marañón', Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona (UAB), CHU Bordeaux [Bordeaux], University of Chemistry and Technology Prague (UCT Prague), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Moscow State University, Université technique de Kazan, Partenaires INRAE, Université Pierre et Marie Curie - Paris 6 (UPMC), University of Bamberg, Fonds d'Investissment pour la Recherche (FIRS) of the Centre Hospitalier Universitaire de Liege, Università degli Studi dell'Aquila = University of L'Aquila (UNIVAQ), University Hospital Erlangen = Uniklinikum Erlangen, Università degli studi di Genova = University of Genoa (UniGe), Internal Medicine, and Pediatrics

Subjects
Adenoma, Male, Cancer Research, medicine.medical_specialty, Endocrinology, Diabetes and Metabolism, Treatment duration, Large population, 030209 endocrinology & metabolism, predictor, Octreotide, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Endocrinology, Internal medicine, Acromegaly, medicine, Humans, Insulin-Like Growth Factor I, MRI, Predictor, Somatostatin analogs, Treatment response, Female, Growth Hormone-Secreting Pituitary Adenoma, Human Growth Hormone, Middle Aged, Prognosis, Somatostatin, Treatment Outcome, Tumor Burden, Magnetic Resonance Imaging, Oncology, business.industry, treatment response, [SDV.MHEP.EM]Life Sciences [q-bio]/Human health and pathology/Endocrinology and metabolism, medicine.disease, somatostatin analogs, Diabetes and Metabolism, Multicenter study, 030220 oncology & carcinogenesis, acromegaly, Signal intensity, business, T2 weighted, Hormone
Abstract

GH-secreting pituitary adenomas can be hypo-, iso- or hyper-intense on T2-weighted MRI sequences. We conducted the current multicenter study in a large population of patients with acromegaly to analyze the relationship between T2-weighted signal intensity on diagnostic MRI and hormonal and tumoral responses to somatostatin analogs (SSA) as primary monotherapy. Acromegaly patients receiving primary SSA for at least 3 months were included in the study. Hormonal, clinical and general MRI assessments were performed and assessed centrally. We included 120 patients with acromegaly. At diagnosis, 84, 17 and 19 tumors were T2-hypo-, iso- and hyper-intense, respectively. SSA treatment duration, cumulative and mean monthly doses were similar in the three groups. Patients with T2-hypo-intense adenomas had median SSA-induced decreases in GH and IGF-1 of 88% and 59% respectively, which were significantly greater than the decreases observed in the T2-iso- and hyper-intense groups (P P P r = 0.22), IGF-1 (P r = 0.14) and adenoma volume (P r = 0.33). The T2-weighted signal intensity of GH-secreting adenomas at diagnosis correlates with hormone reduction and tumor shrinkage in response to primary SSA treatment in acromegaly. This study supports its use as a generally available predictive tool at diagnosis that could help to guide subsequent treatment choices in acromegaly.

Published
2016
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3. McCune-Albright Syndrome: A Detailed Pathological And Genetic Analysis of Disease Effects in an Adult Patient

Author

Albert Thiry, Monique Silvy, Liliya Rostomyan, Patrick Petrossians, Adrian Daly, Anne Barlier, Albert Beckers, Alain Enjalbert, Frédéric Fina, Vladimir Vasilev, Centre de Recherches en Oncologie biologique et Oncopharmacologie (CRO2), Institut National de la Santé et de la Recherche Médicale (INSERM)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Aix Marseille Université (AMU), Anthropologie bio-culturelle, Droit, Ethique et Santé (ADES), Aix Marseille Université (AMU)-EFS ALPES MEDITERRANEE-Centre National de la Recherche Scientifique (CNRS), Etablissement Français du Sang - Alpes-Méditerranée (EFS - Alpes-Méditerranée), Etablissement Français du Sang, Centre de recherche en neurobiologie - neurophysiologie de Marseille (CRN2M), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service d'Endocrinologie (LIEGE - Endocrino), Université de Liège, and Aix Marseille Université (AMU)- Hôpital de la Timone [CHU - APHM] (TIMONE)-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
Adult, Male, medicine.medical_specialty, Pathology, Endocrinology, Diabetes and Metabolism, [SDV]Life Sciences [q-bio], Clinical Biochemistry, 030209 endocrinology & metabolism, Thymus Gland, Fibrous Dysplasia, Polyostotic, Biochemistry, McCune–Albright syndrome, Islets of Langerhans, 03 medical and health sciences, Fatal Outcome, 0302 clinical medicine, Endocrinology, Hypogonadotropic hypogonadism, Internal medicine, Chromogranins, GTP-Binding Protein alpha Subunits, Gs, medicine, GNAS complex locus, Humans, Polyostotic fibrous dysplasia, Pathological, 030304 developmental biology, 0303 health sciences, biology, Mosaicism, business.industry, Hyperparathyroidism, Fibrous dysplasia, Biochemistry (medical), medicine.disease, Neurosecretory Systems, 3. Good health, Gigantism, Radiography, biology.protein, business, Primary hyperparathyroidism
Abstract

McCune Albright syndrome (MAS) is a clinical association of endocrine and nonendocrine anomalies caused by postzygotic mutation of the GNAS1 gene, leading to somatic activation of the stimulatory α-subunit of G protein (Gsα). Important advances have been made recently in describing pathological characteristics of many MAS-affected tissues, particularly pituitary, testicular, and adrenal disease. Other rarer disease related features are emerging.The objective of the investigation was to study the pathological and genetic findings of MAS on a tissue-by-tissue basis in classically and nonclassically affected tissues.This was a comprehensive autopsy and genetic analysis.The study was conducted at a tertiary referral university hospital.An adult male patient with MAS and severe disease burden including gigantism was the subject of the study.Interventions included clinical, hormonal, and radiographic studies and gross and microscopic pathology analyses, conventional PCR, and droplet digital PCR analyses of affected and nonaffected tissues.Pathological findings and the presence of GNAS1 mutations were measured.The patient was diagnosed with MAS syndrome at 6 years of age based on the association of café-au-lait spots and radiological signs of polyostotic fibrous dysplasia. Gigantism developed and hyperprolactinemia, hypogonadotropic hypogonadism, and hyperparathyroidism were diagnosed throughout the adult period. The patient died at the age of 39 years from a pulmonary embolism. A detailed study revealed mosaiscism for the p.R201C GNAS1 mutation distributed across many endocrine and nonendocrine tissues. These genetically implicated tissues included rare or previously undescribed disease associations including primary hyperparathyroidism and hyperplasia of the thymus and endocrine pancreas.This comprehensive pathological study of a single patient highlights the complex clinical profile of MAS and illustrates important advances in understanding the characteristics of somatic GNAS1-related pathology across a wide range of affected organs.

Published
2014
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4. Diabetes in acromegaly, prevalence, risk factors, and evolution: data from the French Acromegaly Registry

Author

Sandrine Fieffe, Isabelle Morange, Patrick Petrossians, Philippe Chanson, Vincent Rohmer, Christine Cortet, Françoise Borson-Chazot, Thierry Brue, Brigitte Delemer, Hôpital Robert Debré, Hôpital Robert Debré-Centre Hospitalier Universitaire de Reims (CHU Reims), CHU de Liège (Endocrinologie), service d'endocrinologie, Récepteurs stéroïdiens : physiopathologie endocrinienne et métabolique, Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR93-Université Paris-Sud - Paris 11 (UP11), E04, Centre de Recherche en Cancérologie de Lyon (UNICANCER/CRCL), Centre Léon Bérard [Lyon]-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Léon Bérard [Lyon]-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de recherche en neurobiologie - neurophysiologie de Marseille (CRN2M), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service d'Endocrinologie - Diabète - Nutrition [Reims], Université de Reims Champagne-Ardenne (URCA)-Hôpital Robert Debré-Centre Hospitalier Universitaire de Reims (CHU Reims), Université Paris-Sud - Paris 11 (UP11)-IFR93-Institut National de la Santé et de la Recherche Médicale (INSERM), and Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)

Subjects
Male, Databases, Factual, MESH: Registries, Endocrinology, Diabetes and Metabolism, MESH: Insulin-Like Growth Factor I, Disease, 030204 cardiovascular system & hematology, MESH: Hypertension, Body Mass Index, MESH: Magnetic Resonance Imaging, 0302 clinical medicine, Endocrinology, MESH: Aged, 80 and over, Risk Factors, MESH: Risk Factors, MESH: Child, Epidemiology, MESH: Human Growth Hormone, Registries, Insulin-Like Growth Factor I, Young adult, Child, Aged, 80 and over, 2. Zero hunger, MESH: Aged, education.field_of_study, MESH: Middle Aged, Human Growth Hormone, General Medicine, MESH: Follow-Up Studies, Middle Aged, Magnetic Resonance Imaging, 3. Good health, MESH: Diabetes Complications, MESH: Young Adult, Hypertension, Disease Progression, Female, MESH: Disease Progression, France, Adult, medicine.medical_specialty, Adolescent, Population, 030209 endocrinology & metabolism, [SDV.CAN]Life Sciences [q-bio]/Cancer, MESH: Multivariate Analysis, MESH: Body Mass Index, Diabetes Complications, MESH: Sex Ratio, Young Adult, 03 medical and health sciences, Internal medicine, Diabetes mellitus, Acromegaly, medicine, Humans, Pituitary Neoplasms, Sex Ratio, Risk factor, MESH: Pituitary Neoplasms, education, Aged, MESH: Adolescent, MESH: Humans, business.industry, MESH: Biological Markers, MESH: Adult, medicine.disease, MESH: Databases, Factual, MESH: Male, MESH: France, Multivariate Analysis, business, Body mass index, MESH: Acromegaly, MESH: Female, Biomarkers, Follow-Up Studies
Abstract

ObjectivesThe French Acromegaly Registry records data of acromegalic patients' since 1992 in French, Belgian (Liège), and Swiss (Lausanne) centers. We studied the prevalence of diabetes in this population looking for risk factors. Patients from one of the centers (Reims) were then analyzed more thoroughly.MethodsThis study has been conducted on all the patients recorded from 1999 until 2004 (519 patients). Evolution of cohorts' was reassessed in 2009. Of the different variables recorded in the registry: age, sex, body mass index (BMI), duration of acromegaly, GH, IGF1 and prolactin levels, pituitary tumor size, hormonal deficiencies, presence, duration and treatment of diabetes, hypertension, and rheumatological disease were analyzed.ResultsThe prevalence of diabetes in the registry was 22.3%. Diabetic patients were older and had a higher BMI. Compared with the data of the French Social Security, acromegalic patients showed a more precocious apparition of diabetes and prevalence was higher in each age group.Compared with non-diabetic acromegalic subjects, diabetic patients had a more prolonged evolution of acromegaly before diagnosis. The levels of GH and IGF1 were not significantly different between the two groups. Only hypertension was significantly more frequent in diabetic patients.ConclusionsIn our population, the prevalence of diabetes was estimated to be 22.3%. The GH and IGF1 levels did not appear as predictive factors for the presence of diabetes. On the contrary, age, BMI, and hypertension were significant risk factors as in the general population of type 2 diabetics.

Published
2011
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5. Clinical characteristics and therapeutic responses in patients with germ-line AIP mutations and pituitary adenomas: an international collaborative study

Author

Robert J. Weil, Timo Sane, Philippe Emy, Carmen Fajardo Montañana, Thierry Brue, Vincent Bours, Cristina L. Ronchi, Tapani Ebeling, Maria Yaneva, Wouter W. de Herder, Mirtha Guitelman, Arnaud Murat, Marie-Thérèse Hagelstein, Laure Cazabat, Dominique Maiter, Fergus J. Cameron, Jérôme Bertherat, Chiara Villa, Sergio P. A. Toledo, Renato Cozzi, Carola Saloranta, Françoise Borson-Chazot, Ian M. Holdaway, Marianthi Georgitsi, Anna Spada, Sebastian J C M M Neggers, Ángel Ferrández Longás, Philippe Chanson, Marie Lise Jaffrain-Rea, Maria Stefania Lagonigro, Albert Beckers, Gustavo Barcelos Barra, Georges Halaby, Sabina Zacharieva, Outi Vierimaa, AntoineAntoine Tabarin, Juliet Jennings, Maria Isabel Sabaté, Elina Heliövaara, Anne Barlier, Antti Raappana, Luciana Ansaneli Naves, Rodrigo A. Toledo, Ernesto De Menis, Günter K. Stalla, José Ignacio Labarta Aizpún, Flavia Magri, Jean-Francis Vanbellinghen, Vinciane Corman, Lauri A. Aaltonen, Pasi I. Salmela, Matti Välimäki, Elisa Verrua, Roberto Salvatori, Eija Eloranta, Anne-Paule Gimenez-Roqueplo, Auli Karhu, Marc Popelier, Adrian Daly, Maria A. Tichomirowa, Patrick Petrossians, Gérald Raverot, Ralf Paschke, Centre de recherche en neurobiologie - neurophysiologie de Marseille (CRN2M), Aix Marseille Université (AMU) - Institut National de la Santé et de la Recherche Médicale (INSERM) - Centre National de la Recherche Scientifique (CNRS), Endocrinology, Cliniques Universitaires Saint-Luc [Bruxelles], Endocrine Genetics Unit (LIM-25), Endocrinology, Department of Internal Medicine, Hospital das Clinicas, University of Sao Paulo School of Medicine, Centre de Psychiatrie et Neurosciences (CPN - U894), Université Paris Descartes - Paris 5 (UPD5) - Institut National de la Santé et de la Recherche Médicale (INSERM), Department of neuropathology, Hôpial Sainte-Anne, Pharmacologie Endocrinienne, Hôpital Lariboisière, Department for Internal Medicine, Endocrinology and Nephrology, University of Leipzig, Pôle Endocrinologie-Diabétologie Adultes-Enfants, Assistance publique - Hôpitaux de Paris (AP-HP) - CHU Cochin [AP-HP] - Hôpital Saint-Vincent de Paul, Récepteurs stéroïdiens : physiopathologie endocrinienne et métabolique, Université Paris-Sud - Paris 11 (UP11) - IFR93 - Institut National de la Santé et de la Recherche Médicale (INSERM), Service d'Endocrinologie et Maladies de la reproduction, Assistance publique - Hôpitaux de Paris (AP-HP) - Hôpital Bicêtre, Internal Medicine, Cell biology, Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Institut de psychiatrie et neurosciences (U894 / UMS 1266), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hôpital Lariboisière-Fernand-Widal [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Universität Leipzig [Leipzig], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Cochin [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Saint-Vincent de Paul, Institut National de la Santé et de la Recherche Médicale (INSERM)-IFR93-Université Paris-Sud - Paris 11 (UP11), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Centre de Psychiatrie et Neurosciences (U894), Universität Leipzig, Université Paris-Sud - Paris 11 (UP11)-IFR93-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Cochin [AP-HP]-Hôpital Saint-Vincent de Paul, and Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Bicêtre

Subjects
Male, Pathology, endocrine system diseases, MESH : Germ-Line Mutation, Endocrinology, Diabetes and Metabolism, Clinical Biochemistry, Adenomi ipofisari, medicine.disease_cause, Biochemistry, Germline, 0302 clinical medicine, Endocrinology, MESH: Germ-Line Mutation, MESH : Female, Multiple endocrine neoplasia, MESH: Dopamine Agonists, Pituitary adenomas, MESH: Treatment Outcome, Mutation, Age Factors, MESH : Pituitary Neoplasms, [SDV.BBM.BC]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Biomolecules [q-bio.BM], 3. Good health, Treatment Outcome, 030220 oncology & carcinogenesis, Dopamine Agonists, Female, Adenoma, medicine.medical_specialty, AIP mutations, MESH : Male, 030209 endocrinology & metabolism, MESH : Treatment Outcome, Biology, AIP "Aryl hydrocarbon receptor Interacting Protein", 03 medical and health sciences, [SDV.BBM.BC] Life Sciences [q-bio]/Biochemistry, Molecular Biology/Biomolecules [q-bio.BM], Germline mutation, MESH : Dopamine Agonists, Pituitary adenoma, Internal medicine, Acromegaly, medicine, gene AIP "Aryl hydrocarbon receptor Interacting Protein", mutazioni del gene AIP, Humans, Pituitary Neoplasms, MESH: Pituitary Neoplasms, [SDV.BBM.BC]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Biochemistry [q-bio.BM], Germ-Line Mutation, MESH: Age Factors, MESH: Adenoma, MESH: Humans, Biochemistry (medical), MESH : Humans, medicine.disease, digestive system diseases, Prolactin, MESH: Male, stomatognathic diseases, MESH : Adenoma, MESH : Age Factors, MESH: Female
Abstract

Context: AIP mutations (AIPmut) give rise to a pituitary adenoma predisposition that occurs in familial isolated pituitary adenomas and less often in sporadic cases. The clinical and therapeutic features of AIPmut-associated pituitary adenomas have not been studied comprehensively. Objective: The objective of the study was to assess clinical/therapeutic characteristics of AIPmut pituitary adenomas. Design: This study was an international, multicenter, retrospective case collection/database analysis. Setting: The study was conducted at 36 tertiary referral endocrine and clinical genetics departments. Patients: Patients included 96 patients with germline AIPmut and pituitary adenomas and 232 matched AIPmut-negative acromegaly controls. Results: The AIPmut population was predominantly young and male (63.5%); first symptoms occurred as children/adolescents in 50%. At diagnosis, most tumors were macroadenomas (93.3%); extension and invasion was common. Somatotropinomas comprised 78.1% of the cohort; there were also prolactinomas (n = 13), nonsecreting adenomas (n = 7), and a TSH-secreting adenoma. AIPmut somatotropinomas were larger (P = 0.00026), with higher GH levels (P = 0.00068), more frequent extension (P = 0.018) and prolactin cosecretion (P = 0.00023), and occurred 2 decades before controls (P < 0.000001). Gigantism was more common in the AIPmut group (P < 0.000001). AIPmut somatotropinoma patients underwent more surgical interventions (P = 0.00069) and had lower decreases in GH (P = 0.00037) and IGF-I (P = 0.028) and less tumor shrinkage with somatostatin analogs (P < 0.00001) vs. controls. AIPmut prolactinomas occurred generally in young males and frequently required surgery or radiotherapy. Conclusions: AIPmut pituitary adenomas have clinical features that may negatively impact treatment efficacy. Predisposition for aggressive disease in young patients, often in a familial setting, suggests that earlier diagnosis of AIPmut pituitary adenomas may have clinical utility. (J Clin Endocrinol Metab 95: E373-E383, 2010)

Published
2010
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