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Outcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2: an international retrospective population-based study

Authordc.contributor.authorCastinetti, Frederic 
Authordc.contributor.authorQi, Xiao-Ping es_CL
Authordc.contributor.authorWalz, Martin K. es_CL
Authordc.contributor.authorMaia, Ana Luiza es_CL
Authordc.contributor.authorSansó, Gabriela es_CL
Authordc.contributor.authorPeczkowska, Mariola es_CL
Authordc.contributor.authorHasse Lazar, Kornelia es_CL
Authordc.contributor.authorLinks, Thera P. es_CL
Authordc.contributor.authorDvorakova, Sarka es_CL
Authordc.contributor.authorToledo, Rodrigo A. es_CL
Authordc.contributor.authorMian, Caterina es_CL
Authordc.contributor.authorBugalho, Maria Joao es_CL
Authordc.contributor.authorWohllk González, Nelson es_CL
Authordc.contributor.authorKollyukh, Oleg es_CL
Authordc.contributor.authorCanu, Letizia es_CL
Authordc.contributor.authorLoli, Paola es_CL
Authordc.contributor.authorBergmann, Simona R. es_CL
Authordc.contributor.authorBiarnes Costa, Josefina es_CL
Authordc.contributor.authorMakay, Ozer es_CL
Authordc.contributor.authorPatocs, Attila es_CL
Authordc.contributor.authorPfeifer, Marija es_CL
Authordc.contributor.authorShah, Nalini S. es_CL
Authordc.contributor.authorCuny, Thomas es_CL
Authordc.contributor.authorBrauckhoff, Michael es_CL
Authordc.contributor.authorBausch, Birke es_CL
Authordc.contributor.authorVon Dobschuetz, Ernst es_CL
Authordc.contributor.authorLetizia, Claudio es_CL
Authordc.contributor.authorBarczynski, Marcin es_CL
Authordc.contributor.authorAlevizaki, Maria K. es_CL
Authordc.contributor.authorCzetwertynska, Malgorzata es_CL
Authordc.contributor.authorUgurlu, M. Umit es_CL
Authordc.contributor.authorValk, Gerlof es_CL
Authordc.contributor.authorPlukker, John T. M. es_CL
Authordc.contributor.authorSartorato, Paola es_CL
Authordc.contributor.authorSiqueira, Debora R. es_CL
Authordc.contributor.authorBarontini, Marta es_CL
Authordc.contributor.authorSzperl, Malgorzata es_CL
Authordc.contributor.authorJarzab, Barbara es_CL
Authordc.contributor.authorVerbeek, Hans H. G. es_CL
Authordc.contributor.authorZelinka, Tomas es_CL
Authordc.contributor.authorVlcek, Petr es_CL
Authordc.contributor.authorToledo, Sergio P. A. es_CL
Authordc.contributor.authorCoutinho, Flavia L. es_CL
Authordc.contributor.authorMannelli, Massimo es_CL
Authordc.contributor.authorRecasens, Mónica es_CL
Authordc.contributor.authorDemarquet, Lea es_CL
Authordc.contributor.authorPetramala, Luigi es_CL
Authordc.contributor.authorYaremchuk, Svetlana es_CL
Authordc.contributor.authorZabolotnyi, Dmitry es_CL
Authordc.contributor.authorSchiavi, Francesca es_CL
Authordc.contributor.authorOpocher, Giuseppe es_CL
Authordc.contributor.authorRacz, Karoly es_CL
Authordc.contributor.authorJanuszewicz, Andrzej es_CL
Authordc.contributor.authorWeryha, Georges es_CL
Authordc.contributor.authorHenry, Jean Francois es_CL
Authordc.contributor.authorBrue, Thierry es_CL
Authordc.contributor.authorConte Devolx, Bernard es_CL
Authordc.contributor.authorEng, Charis es_CL
Authordc.contributor.authorNeumann, Hartmut P. H. es_CL
Admission datedc.date.accessioned2014-12-16T18:18:30Z
Available datedc.date.available2014-12-16T18:18:30Z
Publication datedc.date.issued2014
Cita de ítemdc.identifier.citationLancet Oncol 2014; 15: 648–55en_US
Identifierdc.identifier.otherdx.doi.org/10.1016/ S1470-2045(14)70154-8
Identifierdc.identifier.urihttp://repositorio.uchile.cl/handle/2250/129398
General notedc.descriptionArtículo de publicación ISIen_US
Abstractdc.description.abstractBackground The prevention of medullary thyroid cancer in patients with multiple endocrine neoplasia type 2 syndrome has demonstrated the ability of molecular diagnosis and prophylactic surgery to improve patient outcomes. However, the other major neoplasia associated with multiple endocrine neoplasia type 2, phaeochromocytoma, is not as well characterised in terms of occurrence and treatment outcomes. In this study, we aimed to systematically characterise the outcomes of management of phaeochromocytoma associated with multiple endocrine neoplasia type 2. Methods This multinational observational retrospective population-based study compiled data on patients with multiple endocrine neoplasia type 2 from 30 academic medical centres across Europe, the Americas, and Asia. Patients were included if they were carriers of germline pathogenic mutations of the RET gene, or were fi rst-degree relatives with histologically proven medullary thyroid cancer and phaeochromocytoma. We gathered clinical information about patients’ RET genotype, type of treatment for phaeochromocytoma (ie, unilateral or bilateral operations as adrenalectomy or adrenal-sparing surgery, and as open or endoscopic operations), and postoperative outcomes (adrenal function, malignancy, and death). The type of surgery was decided by each investigator and the timing of surgery was patient driven. The primary aim of our analysis was to compare disease-free survival after either adrenal-sparing surgery or adrenalectomy. Findings 1210 patients with multiple endocrine neoplasia type 2 were included in our database, 563 of whom had phaeochromocytoma. Treatment was adrenalectomy in 438 (79%) of 552 operated patients, and adrenal-sparing surgery in 114 (21%). Phaeochromocytoma recurrence occurred in four (3%) of 153 of the operated glands after adrenal-sparing surgery after 6–13 years, compared with 11 (2%) of 717 glands operated by adrenalectomy (p=0·57). Postoperative adrenal insuffi ciency or steroid dependency developed in 292 (86%) of 339 patients with bilateral phaeochromocytoma who underwent surgery. However, 47 (57%) of 82 patients with bilateral phaeochromocytoma who underwent adrenal-sparing surgery did not become steroid dependent. Interpretation The treatment of multiple endocrine neoplasia type 2-related phaeochromocytoma continues to rely on adrenalectomies with their associated Addisonian-like complications and consequent lifelong dependency on steroids. Adrenal-sparing surgery, a highly successful treatment option in experienced centres, should be the surgical approach of choice to reduce these complications. Funding European Union, German Cancer Foundation, Arthur Blank Foundation, Italian Government, Charles University, Czech Ministry of Health, Nanjing Military Command, National Science Centre Poland, National Research Council for Scientifi c and Technological Development, and State of São Paulo Research Foundation.en_US
Patrocinadordc.description.sponsorshipThe study was supported in part by grants from the European Union (grant LSHC-CT-2005-518200); the German Cancer Foundation (grant 107995 to HPHN); the Arthur Blank Foundation in Atlanta, GA, USA (to CE); the Italian Government project on rare diseases (grant 10FF11 to MM); the research programme of Charles University (P27/LF1/1) and a research grant from the Czech Ministry of Health (NT12336-4/2011) (to TZ); the Key Scientifi c Research Project of Nanjing Military Command, China (09Z038 and 10Z036 to X-PQ); the National Science Centre Poland (grant N401 410639 to KH-L and BJ); the National Research Council for Scientifi c and Technological Development grant 401990/2010-9; the State of São Paulo Research Foundation (FAPESP) grants 2009/11942-1, 11/13518-2, and 13/01476-9 (to RAT, SPAT, and FLC), and grants IGA MH CZ NT/13901-4 and MH CZ 00023761 (to SD). CE is the Sondra J and Stephen R Hardis Endowed Chair in Cancer Genomic Medicine at the Cleveland Clinic (OH, USA) and is an American Cancer Society Clinical Research Professor. SPAT is a CAPES visiting senior professor at the Federal University State of São Paulo (UNIFESP).en_US
Lenguagedc.language.isoenen_US
Publisherdc.publisherElsevieren_US
Type of licensedc.rightsAttribution-NonCommercial-NoDerivs 3.0 Chile*
Link to Licensedc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/3.0/cl/*
Títulodc.titleOutcomes of adrenal-sparing surgery or total adrenalectomy in phaeochromocytoma associated with multiple endocrine neoplasia type 2: an international retrospective population-based studyen_US
Document typedc.typeArtículo de revistaen_US


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Except where otherwise noted, this item's license is described as Attribution-NonCommercial-NoDerivs 3.0 Chile