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The early diagnosis and\ninitial normative treatment are helpful for the long-term outcome of MEN2A.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Methods:<\/jats:title>\n<jats:p>Three index cases and their 29 relatives from three families with MEN2A were included\nin this study. Genetic screening was performed on all participants. Demographic, clinical profiles,\ntumor histopathologic features, and follow-up records were systematically analyzed.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Results:<\/jats:title>\n<jats:p>In total, RET C634Y mutation was identified in 10 individuals (10\/32, 31.3%). Among\nthem, 5 presented with MTC symptoms, whereas the other 5 did not show apparent clinical manifestation,\nand all were subjected to thyroidectomy with varying neck dissection. Compared to individuals\nin the former, the latter benefited greatly from RET screening with significantly younger\nage at diagnosis of MTC and surgery (18.1 \u00b1 13.8 years vs. 39.0 \u00b1 14.1 years, P =0.045), and lessaggressive\nMTC behavior (size: 0.74 vs. 2.82 cm, P =0.026; LN+\/resected: 20.0% vs. 100.0%, P\n=0.048) and also lower recurrence rate of MTC (20.0% vs. 100.0%, P =0.048). The PHEO was\nidentified in 6 of the 10 carriers (60.0%), and all had undergone adrenal-sparing surgery. During\nthe 10 years of follow-up, one (16.7%) developed recurrence of PHEO.<\/jats:p>\n<\/jats:sec>\n<jats:sec>\n<jats:title>Conclusion:<\/jats:title>\n<jats:p>Integrated RET screening, serum calcitonin, and plasma metanephrine\/ normetanephrine\nlevels can facilitate the early diagnosis and standardized MTC\/PHEO surgery to improve\nthe prognosis of MEN2A. Laparoscopic adrenal-sparing surgery prior to the bilateral total\nthyroidectomy is a preferred surgical approach for PHEO.<\/jats:p>\n<\/jats:sec>","DOI":"10.2174\/2214083203666210826094602","type":"journal-article","created":{"date-parts":[[2021,8,27]],"date-time":"2021-08-27T05:26:16Z","timestamp":1630041976000},"page":"339-347","update-policy":"https:\/\/doi.org\/10.2174\/bsp_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Impact of RET Screening on the Management of Multiple Endocrine Neoplasia\nType 2A: 10 Years Experience and Follow-Up in Three Families"],"prefix":"10.2174","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6456-4310","authenticated-orcid":true,"given":"Yue-Ping","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Urology, The Affiliated Jinhua Hospital, Zhejiang University School of Medicine, 365 Renmin East\nRoad, Jinhua 321000, Zhejiang Province, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9427-6643","authenticated-orcid":true,"given":"Fei-Ping","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Urology, The Taizhou Hospital of Zhejiang Province\nAffiliated to Wenzhou Medical University, 150 Ximen Street, Linhai 317000, Zhenjiang Province, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0663-2119","authenticated-orcid":true,"given":"Hui-Hong","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Oncologic and Urologic Surgery, The 903rd PLA Hospital, Wenzhou Medical University, 40 Jichang Road,\nHangzhou 310004, Zhejiang Province, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4272-7940","authenticated-orcid":true,"given":"Xu-Dong","family":"Fang","sequence":"additional","affiliation":[{"name":"Department of Oncologic and Urologic Surgery, The 903rd PLA Hospital, Wenzhou Medical University, 40 Jichang Road,\nHangzhou 310004, Zhejiang Province, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4614-9479","authenticated-orcid":true,"given":"Zai-Sheng","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Urology, The Affiliated Jinhua Hospital, Zhejiang University School of Medicine, 365 Renmin East\nRoad, Jinhua 321000, Zhejiang Province, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yong-Liang","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Urology, Shaoxing Central Hospital, China Medical\nUniversity, 1 Huayu Road, Keqiao District, Shaoxing 312030, Zhejiang Province, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1543-0727","authenticated-orcid":true,"given":"Xiao-Ping","family":"Qi","sequence":"additional","affiliation":[{"name":"Department of Oncologic and Urologic Surgery, The 903rd PLA Hospital, Wenzhou Medical University, 40 Jichang Road,\nHangzhou 310004, Zhejiang Province, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"965","reference":[{"key":"ref=1","doi-asserted-by":"publisher","first-page":"3149","DOI":"10.1210\/jc.2013-1204","volume":"98","author":"Wells S.A.","year":"2013","unstructured":"Wells S.A.; Pacini F.; Robinson B.G.; Santoro M.; Multiple endocrine neoplasia type 2 and familial medullary thyroid carcinoma: An update. 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