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     <dc:title xml:lang="fr">Complexe de Carney, étude des lésions mammaires dans une cohorte prospective de 70 patients : est-ce un syndrome prédisposant au cancer du sein ?</dc:title>
     <dcterms:alternative xml:lang="en">Carney complex, study of breast lesions in a prospective cohort of 70 patients : is it a predisposing syndrome for breast cancer ?</dcterms:alternative>
     <dc:subject xml:lang="fr">Complexe de Carney</dc:subject><dc:subject xml:lang="fr">PRKAR1A</dc:subject><dc:subject xml:lang="fr">carcinome mammaire</dc:subject><dc:subject xml:lang="fr">lésion mammaire</dc:subject>
     <dc:subject xml:lang="en">Carney Complex</dc:subject><dc:subject xml:lang="en">PRKAR1A</dc:subject><dc:subject xml:lang="en">breast carcinoma</dc:subject><dc:subject xml:lang="en">breast lesion</dc:subject><tef:sujetRameau><tef:vedetteRameauNomCommun>
						<tef:elementdEntree autoriteSource="Sudoc" autoriteExterne="027389847">Maladies héréditaires</tef:elementdEntree>
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						<tef:elementdEntree autoriteSource="Sudoc" autoriteExterne="027248585">Sein‎--Cancer</tef:elementdEntree>
					</tef:vedetteRameauNomCommun><tef:vedetteRameauNomCommun>
						<tef:elementdEntree autoriteSource="Sudoc" autoriteExterne="027314014">Sein</tef:elementdEntree><tef:subdivision autoriteSource="Sudoc" type="subdivisionDeSujet" autoriteExterne="027658392">Lésions et blessures </tef:subdivision>
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     <dcterms:abstract xml:lang="fr">Le complexe de Carney (CNC), regroupe des néoplasies endocriniennes ou non, dont des atteintes mammaires, primordiales à étudier, au vu de la fréquence et létalité du carcinome mammaire. A partir d'une cohorte de 70 patients atteints de CNC, dont le génotype et les manifestations cliniques ont été recueillies, une analyse des lésions mammaires et leur prise en charge a été menée chez 50 femmes. Parmi les 38 femmes avec imagerie mammaire, dix présentaient des lésions bénignes et six des carcinomes (un carcinome in-situ, cinq invasifs, une récurrence controlatérale). La survenue de cancer mammaire en contexte de CNC était plus fréquente qu'en population générale avec un âge moyen 17 ans plus précoce (44,7 ans). Tous les cancers mammaires survenaient chez des patientes mutées PRKAR1A, avec de bons facteurs pronostic. Il n’y avait pas d’association entre les carcinomes mammaires et les manifestations du CNC ou les facteurs de risque conventionnels. Les lésions mammaires sont fréquentes dans les femmes avec CNC et le carcinome mammaire pourrait être une « nouvelle » manifestation de ce syndrome.</dcterms:abstract>
     <dcterms:abstract xml:lang="en">The Carney complex (CNC), brings together endocrine and non-endocrine neoplasia, including breast lesions, which are essential to study, given the frequency and lethality of breast carcinoma. From a cohort of 70 patients with CNC, whose genotype and clinical manifestations have been collected, an analysis of breast lesions and their management was carried out in 50 women. Among the 38 women with breast imaging, ten presented benign lesions and six had carcinomas (one in situ, five invasive, one contralateral recurrence). The frequency of breast cancer in patients with CNC was higher and the average age at diagnosis was 17 years earlier (44.7 years) than the general population. All breast cancers occurred in PRKAR1A mutated patients, with good prognostic factors. There was no association between breast carcinomas and manifestations of CNC or conventional risk factors. Breast lesions are common in women with CNC and breast carcinoma may be a "new" manifestation of this syndrome.</dcterms:abstract>
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       <tef:nom>Vaduva</tef:nom>
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