Ectopic ACTH syndrome in children and adolescents
| Autor: | More, Julie, Young, Jacques, Reznik, Yves, Raverot, Gérald, Borson-Chazot, Françoise, Rohmer, Vincent, Baudin, Eric, Coutant, Régis, Tabarin, Antoine, Renseigné, Non |
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| Přispěvatelé: | Service d'endocrinologie, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Bicêtre, Cancers et préventions, Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), 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), Université d'Angers (UA), Institut Gustave Roussy (IGR), Médecine nucléaire, Département d'imagerie médicale [Gustave Roussy], Institut Gustave Roussy (IGR)-Institut Gustave Roussy (IGR), Endocrinologie pédiatrique, Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Département d'endocrinologie - Bordeaux 2, Université Bordeaux Segalen - Bordeaux 2 |
| Rok vydání: | 2011 |
| Předmět: |
Male
Hydrocortisone Endocrinology Diabetes and Metabolism Clinical Biochemistry Growth Weight Gain Biochemistry Dexamethasone MESH: Magnetic Resonance Imaging MESH: Cushing Syndrome 0302 clinical medicine Endocrinology MESH: Health Surveys MESH: Pituitary Gland MESH: Child Receptors Somatostatin Child Cushing Syndrome MESH: Treatment Outcome education.field_of_study Outcome measures food and beverages Nutritional status Prognosis Magnetic Resonance Imaging MESH: Hydrocortisone MESH: Positron-Emission Tomography 3. Good health ACTH Syndrome Ectopic ACTH-Secreting Pituitary Adenoma Treatment Outcome 030220 oncology & carcinogenesis MESH: Dexamethasone Pituitary Gland MESH: Weight Gain Female France MESH: ACTH Syndrome Ectopic MESH: Tomography X-Ray Computed hormones hormone substitutes and hormone antagonists medicine.drug endocrine system medicine.medical_specialty Adolescent Population Radioimmunoassay 030209 endocrinology & metabolism [SDV.CAN]Life Sciences [q-bio]/Cancer Adrenocorticotropic hormone MESH: Prognosis MESH: Radioimmunoassay Diagnosis Differential 03 medical and health sciences Adrenocorticotropic Hormone MESH: Diagnosis Differential Internal medicine medicine MESH: Receptors Somatostatin Humans education Pituitary ACTH Hypersecretion MESH: Adrenocorticotropic Hormone MESH: Adolescent MESH: Growth MESH: Humans business.industry MESH: Pituitary ACTH Hypersecretion fungi Biochemistry (medical) Ewing's sarcoma medicine.disease Health Surveys MESH: Male MESH: ACTH-Secreting Pituitary Adenoma MESH: France El Niño Ectopic ACTH syndrome Positron-Emission Tomography business Tomography X-Ray Computed MESH: Female |
| Zdroj: | Journal of Clinical Endocrinology and Metabolism Journal of Clinical Endocrinology and Metabolism, Endocrine Society, 2011, 96 (5), pp.1213-22. ⟨10.1210/jc.2010-2276⟩ |
| ISSN: | 1945-7197 0021-972X |
| Popis: | International audience; CONTEXT: Ectopic ACTH syndrome (EAS) in youngsters has seldom been reported and is poorly known. SETTING: We conducted a multicenter retrospective study involving 18 French tertiary hospitals. Cases of EAS presenting Cushing's syndrome before the age of 20 during the period from 1985 to 2008 were analyzed. PATIENTS: Ten patients aged 14 to 20 yr were identified and compared to 20 age-matched patients with Cushing's disease diagnosed during the same period. MAIN OUTCOME MEASURES: Etiologies, clinical, biochemical and radiological features, prognosis, and treatment were described. RESULTS: Seven patients had well-differentiated neuroendocrine tumors (five bronchial carcinoids, one mediastinal lymph node, and one thymic), one had a poorly differentiated thymic carcinoma, one had a pleural Ewing's sarcoma, and one had a liver nested stromal epithelial tumor. At presentation, seven tumors were identified with computed tomography scanning and somatostatin receptor scintigraphy, and one with fluoro-18-L-dihydroxyphenylalanine positron emission tomography scan. Two carcinoids were occult and were identified during follow-up. Cushing's syndrome was more intense in EAS, but the clinical and biological spectrum overlapped with that of Cushing's disease. No dynamic test achieved 100% accuracy, whereas petrosal sinus sampling provided correct diagnosis in all patients tested. Medical treatment of hypercortisolism was successful in six of the eight patients with whom it was attempted, and bilateral adrenalectomy had to be performed in only two cases. Prognosis was good; nine patients with curative resection of the tumor were alive and cured (median follow-up, 6.5 yr), whereas one patient died. CONCLUSIONS: EAS in youngsters displays many similarities to that described in adults. The diagnostic and therapeutic algorithms recommended in adults can be used in this population. |
| Databáze: | OpenAIRE |
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