{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,30]],"date-time":"2026-06-30T16:46:11Z","timestamp":1782837971783,"version":"3.54.5"},"reference-count":44,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,6,28]],"date-time":"2014-06-28T00:00:00Z","timestamp":1403913600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Clinical Endocrinology"],"published-print":{"date-parts":[[2014,9]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Multiple endocrine neoplasia type 2 (<jats:styled-content style=\"fixed-case\">MEN<\/jats:styled-content>2) is a rare autosomal dominant syndrome caused by mutations in the RET protooncogene and is characterized by a strong penetrance of medullary thyroid carcinoma (all subtypes) and is often accompanied by pheochromocytoma (<jats:styled-content style=\"fixed-case\">MEN<\/jats:styled-content>2A\/2B) and primary hyperparathyroidism (<jats:styled-content style=\"fixed-case\">MEN<\/jats:styled-content>2A). The evaluation and management of <jats:styled-content style=\"fixed-case\">MEN<\/jats:styled-content>2\u2010related tumours is often different from that of sporadic counterparts. This review article provides an overview of clinical manifestations, diagnosis and surgical management of <jats:styled-content style=\"fixed-case\">MEN<\/jats:styled-content>2 patients. This review also presents applications of the most up\u2010to\u2010date imaging modalities to <jats:styled-content style=\"fixed-case\">MEN<\/jats:styled-content>2 patients that are tightly linked to the clinical management and aims to guide physicians towards a rationale for the use of imaging prior to prophylactic thyroidectomy, initial surgery and reoperations for persistent\/recurrent disease. This review also concludes that, in the near future, it is expected that these patients will indeed benefit from newly developed positron emission tomography approaches which will target peptide receptors and protein kinases. Identification of <jats:styled-content style=\"fixed-case\">MEN<\/jats:styled-content>2\u2010specific radiopharmaceuticals will also soon arise from molecular profiling studies. Furthermore, subtotal (cortical\u2010sparing) adrenalectomy, which is a valid option in MEN2 for avoiding long\u2010term steroid replacement, will benefit from an accurate estimation through imaging of differential adrenocortical function.<\/jats:p>","DOI":"10.1111\/cen.12513","type":"journal-article","created":{"date-parts":[[2014,5,30]],"date-time":"2014-05-30T09:13:31Z","timestamp":1401441211000},"page":"317-328","source":"Crossref","is-referenced-by-count":29,"title":["Diagnosis and preoperative imaging of multiple endocrine neoplasia type 2: current status and future directions"],"prefix":"10.1111","volume":"81","author":[{"given":"David","family":"Ta\u00efeb","sequence":"first","affiliation":[{"name":"Department of Nuclear Medicine La Timone University Hospital CERIMED Aix\u2010Marseille University Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Electron","family":"Kebebew","sequence":"additional","affiliation":[{"name":"Endocrine Oncology Branch National Cancer Institute National Institutes of Health Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fr\u00e9deric","family":"Castinetti","sequence":"additional","affiliation":[{"name":"Department of Endocrinology La Timone University Hospital Aix\u2010Marseille University Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clara C.","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Radiology and Imaging Sciences Department Warren Magnuson Clinical Center National Institutes of Health Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jean\u2010Fran\u00e7ois","family":"Henry","sequence":"additional","affiliation":[{"name":"Department of Endocrine Surgery La Timone University Hospital Aix\u2010Marseille University Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karel","family":"Pacak","sequence":"additional","affiliation":[{"name":"Program in Reproductive and Adult Endocrinology Eunice Kennedy Shriver National Institute of Child Health &amp; 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