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临床病理特征和突变谱对甲状腺髓样癌长期生存及复发的不同影响

Distinct Impacts of Clinicopathological and Mutational Profiles on Long-Term Survival and Recurrence in Medullary Thyroid Carcinoma.

作者信息

Oh Moon Young, Jung Kyong Yeun, Choi Hoonsung, Chai Young Jun, Cho Sun Wook, Kim Su-Jin, Lee Kyu Eun, Chung Eun-Jae, Park Do Joon, Park Young Joo, Yang Han-Kwang

机构信息

Department of Surgery, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul National University College of Medicine, Seoul, Korea.

Department of Internal Medicine, Nowon Eulji Medical Center, Eulji University, Seoul, Korea.

出版信息

Endocrinol Metab (Seoul). 2024 Dec;39(6):877-890. doi: 10.3803/EnM.2024.2027. Epub 2024 Nov 5.

Abstract

BACKGRUOUND

Medullary thyroid carcinoma (MTC) has a poorer prognosis than differentiated thyroid cancers; however, comprehensive data on the long-term outcomes of MTC remain scarce. This study investigated the extended clinical outcomes of MTC and aimed to identify prognostic factors.

METHODS

Patients diagnosed with MTC between 1980 and 2020 were retrospectively reviewed. Their clinical characteristics, longterm clinical outcomes, and prognostic factors for recurrence and mortality were analyzed.

RESULTS

The study included 226 patients (144 women, 82 men). The disease-specific survival (DSS) rates for all MTC patients at 5-, 10-, 20-, and 30-year intervals were 92.7%, 89.4%, 74.3%, and 68.1%, respectively. The recurrence-free survival (RFS) rates were 71.1%, 56.1%, 40.2%, and 32.1% at these intervals. DSS was comparable between the groups from 1980-2009 and 2010-2020 (P=0.995); however, the 1980-2009 group had significantly lower RFS rates (P=0.031). The 2010-2020 group exhibited greater extents of surgical and lymph node dissection (P=0.003) and smaller tumors (P=0.003). Multivariate analysis identified extrathyroidal extension as the strongest prognostic factor for both RFS and DSS. Age >55 years and tumor size of ≥2 cm were also significant prognostic factors for DSS, while hereditary disease and lymph node metastasis were significant for RFS. Survival analysis after propensity-score matching of rearranged during transfection (RET)-negative and non-screened RET-positive groups showed comparable DSS but longer RFS in the RET-negative group.

CONCLUSION

Extrathyroidal extension was identified as the strongest prognostic factor for RFS and DSS. Older age and larger tumor size were associated with decreased DSS, while RET mutation and lymph node metastasis significantly impacted RFS.

摘要

背景

髓样甲状腺癌(MTC)的预后比分化型甲状腺癌差;然而,关于MTC长期预后的综合数据仍然稀缺。本研究调查了MTC的扩展临床结局,并旨在确定预后因素。

方法

对1980年至2020年间诊断为MTC的患者进行回顾性分析。分析了他们的临床特征、长期临床结局以及复发和死亡的预后因素。

结果

该研究纳入了226例患者(144例女性,82例男性)。所有MTC患者在5年、10年、20年和30年时的疾病特异性生存率(DSS)分别为92.7%、89.4%、74.3%和68.1%。这些时间点的无复发生存率(RFS)分别为71.1%、56.1%、40.2%和32.1%。1980 - 2009年组和2010 - 2020年组的DSS相当(P = 0.995);然而,1980 - 2009年组的RFS率显著较低(P = 0.031)。2010 - 2020年组的手术和淋巴结清扫范围更大(P = 0.003),肿瘤更小(P = 0.003)。多因素分析确定甲状腺外侵犯是RFS和DSS最强的预后因素。年龄>55岁和肿瘤大小≥2 cm也是DSS的显著预后因素,而遗传性疾病和淋巴结转移对RFS有显著影响。对转染重排(RET)阴性和未筛查的RET阳性组进行倾向得分匹配后的生存分析显示,两组DSS相当,但RET阴性组的RFS更长。

结论

甲状腺外侵犯被确定为RFS和DSS最强的预后因素。年龄较大和肿瘤较大与DSS降低相关,而RET突变和淋巴结转移对RFS有显著影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3b45/11695486/3b1163072f3e/enm-2024-2027f1.jpg

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