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prophylactic thyroidectomy for MEN 2-related medullary thyroid carcinoma based on predictive testing for RET proto-oncogene mutation and basal serum calcitonin in China

Prophylactic thyroidectomy for MEN 2-related medullary thyro...

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2013浙江省医学遗传学学术年会

作者： Xiao-Ping Qi Zhen-Fang Du Ju-Ming Ma Jun Fei Jun Cheng Jian-Shan Han Hang-Yang Jin Zhen-Guang Chen Jin-Quan Wang Xiao-Ling Chen Wen-Ting Liu Yan Zhao Hu-Ling Jiang Xian-Ning Zhang Department of Oncologic Surgery Department of Urologic SurgeryClinical Experimental Centerand Department of PathologyThe 117th PLA Hospital Department of Biochemistry and Genetics National Education Base for Basic Medical SciencesInstitute of Cell BiologyZhejiang University School of Medicine

Introduction:Early and normative surgery is the only curative method for multiple endocrine neoplasia type 2(MEN 2)-related medullary thyroid carcinoma(MTC). Aims:To study the timing of prophylactic total thyroidectomy(TT) for MEN 2-related MTC with different RET mutations in a Chinese population,and to compare the sensitivity and accuracy of fully-automated chemiluminescence immunoassay(FACLIA) and radioimmunoassay(RIA) for serum calcitonin(Ct). Msthods:We collected 24 asymptomatic individuals from 8 unrelated Chinese families with MEN 2,and analyzed RET mutation and Ct *** we performed TT on 17 of the 24 individuals,including TT (2/17),TT with bilateral level VI lymph-node dissection(B-LND(VI);12/17) and TT with B-LND(VI) + modified unilateral/bilateral/local neck dissection(3/17). Results:Histopathology revealed bilateral/unilateral MTC in 15/17(88.2%;median diameter,1.0 cm) and bilateral C-cell hyperplasia in 2/17(11.8%;p.V292M/R67H/R982C and p.C618Y).Lymph-node metastasis/ fibro-adipose tissue invasion(p.C634R) or solely fibro-adipose tissue invasion(p.C634Y) were found in 2/ 17(11.8%).Elevated pre-surgical Ct(pre-Ct) was identified by FACLIA in 17/17(median age,24.0), while pre-Ct by RIA was found in only 6/15(P prophylactic TT for MEN 2-related MTC,based on predictive integrated screening of RET and pre-Ct ***,we recommend FACLIA to measure Ct for earlier diagnosis,treatment and follow-up monitoring of MTC.

关键词： prophylactic thyroidectomy Medullary thyroid carcinoma RET Mutation screening Serum calcitonin Fully-automated chemiluminescence immunoassay

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Is new American Thyroid Association risk classification for hereditary medullary thyroid carcinoma applicable to Chinese patients? A single-center study

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Chinese Journal of Cancer Research 2017年第3期29卷 223-230页

作者： Xiwei Zhang Dangui Yan Junyi Wang Hanfeng Wan Yongxia Zhang Yabing Zhang Yuqin He Wensheng Liu Bin Zhang Department of Head and Neck Surgical Oncology National Cancer Center/Cancer Hospital Chinese Academy of Medical Science and Peking Union Medical College Key Laboratory of Cancer Prevention and Therapy of Tianjin Department of Thyroid and Neck Tumor Tianjin Medical University Cancer Institute and Hospital National Clinical Research Center for Cancer Department of Otorhinolaryngology Head and Neck Surgery Renji Hospital Shanghai Jiao Tong University School of Medicine Department of Otorhinolaryngology Head and Neck Surgery Chinese People’s Liberation Army (PLA) General Hospital Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education/Beijing) Department of Head and Neck Surgical Oncology Peking University Cancer Hospital & Institute

Objective: The American Thyroid Association (ATA) proposed a new risk classification for hereditary medullary thyroid carcinoma (MTC) in 2015. This study aimed to assess whether the new guidelines are suitable for the Chinese population, and reported our experience on prophylactic thyroidectomy. Methods: A total of 73 patients from 22 families were screened as rearranged during transfection (RET) mutation carriers from 2010 to 2016 in Cancer Hospital, Chinese Academy of Medical Science; the medical history for each patient was collected. Based on the initial treatment, we identified the risk factors for poor prognosis by univariate and multivariate logistic regression. Then, 4 RET mutation carriers were enrolled for prophylactic thyroidectomy, and their pathological data and follow-up outcomes were recorded. Results: In univariate and multivariate logistic regression analyses, age at initial surgery and risk classification were significant risk factors for stage III/IV hereditary MTC at initial diagnosis. The likelihood was increased by 11.6% per year of age at initial surgery [95% confidence interval (95% CI), 1.040-1.198; P=0.002). It was 7.888 times more likely to have III/IV stage disease for ATA highest risk patients, compared to ATA moderate risk individuals (95% CI, 1.607-38.717; P=0.003). Postoperative pathological results showed all 4 multiple endocrine neoplasia type 2A (MEN2A) patients had C-cell hyperplasia (CCH); multifocal malignancies were detected in 3 of them. All 4 patients were cured biochemically, and none developed permanent hypoparathyroidism. Conclusions: In Chinese individuals, hereditary MTC aggressiveness is in line with the new ATA risk classification. Germline RET gene mutation carriers should undergo prophylactic thyroidectomy according to basal serum calcitonin levels.

关键词： Medullary thyroid carcinoma (MTC) rearranged during transfection (RET) genotype-phenotype correlation multiple endocrine neoplasia type 2 (MEN2) prophylactic thyroidectomy

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Molecular Diagnosis and Comprehensive Treatment of Multiple Endocrine Neoplasia Type 2 in Southeastern Chinese

Molecular Diagnosis and Comprehensive Treatment of Multiple ...

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2014浙江省医学会医学遗传学学术年会

作者：赵坚强陈振光戚晓平解放军第一一七医院肿瘤泌尿外科浙江省肿瘤医院头颈外科

PURPOSE:To study the feasibility and curative effect of comprehensive clinical management of MEN2-related medullary thyroid carcinoma(MTC)and pheochromocytoma(PHEO)based on systemic family screening and RET ***:We report twenty-six of 33 MEN2 patients who underwent(prophylactic)thyroidectomy with varying neck dissection including one patient therapied by Vandetanib after surgery and eight of 24 MEN2A patients with PHEO underwent cortical sparing adrenalectomy(CSA).Direct sequencing of entire RET exons was performed in all ***:RET mutations were confirmed in 20 symptomatic patients and identified in 13 at-risk relatives(RET-carriers).Of the 26 surgery patients(25 with MTC and 1 with C-cell hyperplasia),the mean age at first thyroid surgery and tumor diameter of 6 RET-carriers was decreased compared with 20 symptomatic patients(P=0.000,P=0.007;respectively),one patient with lymph node metastasis in RET-carriers,compared with nineteen among 20 indexed cases(P=0.0001),and disease-free survival(DFS)increased(DFS:80%vs.10%,P=0.0001).However,seven RET-carriers rejected *** MEN2A-MTC with bone metastases received Vandetanib therapy for 20 months and responded *** MEN2A-PHEOs underwent CSA who initially unilateral disease,one developed contralateral PHEO 10 years later,then also accepted *** needn’t steriod replacement ***:Predictive integrated screening of RET and preoperative calcitonin level is an excellent strategy to ensure earlier diagnosis and standardize thyroidectomy of *** advanced MTC,vandetanib provides novel treatment *** PHEO,CSA can be prioritized to preserve adrenocortical function.

关键词： Multiple endocrine neoplasia type 2 Mutation screening RET prophylactic thyroidectomy Cortical sparing adrenalectomy Tyrosine kinase inhibitor

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Molecular Diagnosis and Comprehensive Treatment of Multiple Endocrine Neoplasia Type 2 in Southeastern Chinese

Molecular Diagnosis and Comprehensive Treatment of Multiple ...

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2015年浙江省泌尿外科学男科学学术年会

作者：陈振光赵坚强戚晓平解放军第一一七医院泌尿外科浙江省肿瘤医院头颈外科

We present a comprehensive genetic and clinical analysis of nine Chinese families with *** RET mutations(p.C634Y[n=10],p.C634R[n=9],p.C634F[n=2],p.C618Y[n=8],p.C618R[n=3],and p.M918T[n=1])were confirmed in 20 symptomatic patients and identified in 13 at-risk relatives(RET carriers).Twenty-six of 33 MEN2 patients underwent thyroidectomies with neck dissections;the mean age at the time of the first thyroid surgery and the tumor diameter of the 6 RET carriers was decreased compared with 20 symptomatic patients(P<0.001 and P=0.007,respectively),while the disease-free survival was increased(80%vs.10%,P=0.0001).Seven RET carriers who were declined *** of 20 symptomatic patients with medullary thyroid cancer(MTC)bone metastases after surgery received vandetanib therapy for 20 months and responded ***,8 of 24 MEN2A patients who initially had unilateral pheochromocytomas underwent cortical-sparing adrenalectomy(CSA),1developed contralateral pheochromo-cytomas 10 years later,then also acceptedand also agreed to a *** of the patients required steroid replacement *** on our results,integrated RET screening and the pre-operative calcitonin level is an excellent strategy to ensure earlier diagnosis and standard *** can be utilized to preserve adrenocortical function in patients with pheochromocytomas.

关键词： multiple endocrine neoplasia type 2 mutation screening RET prophylactic thyroidectomy cortical-sparing adrenalectomy tyrosine kinase inhibitor

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