Alessandro Prete, Liborio Torregrossa, Carla Gambale, Raffaele Ciampi, Teresa Ramone, Cristina Romei, Virginia Cappagli, Paolo Piaggi, Clara Ugolini, Gabriele Materazzi, Rossella Elisei, Antonio Matrone
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We divided patients according to the extent of the disease at diagnosis into localized (183/314, 58.3%) (confined to the thyroid), regional (100/314, 31.8%) (limited to the neck, involving surrounding thyroid tissues and/or regional lymph nodes), and distant (31/314, 9.9%) (distant metastases) disease. Data about somatic mutations were available in 212/314 (67.5%) patients. Expert pathologists differentiated high- and low-grade tumors. <b><i>Results:</i></b> According to the IMTCGS, 115/314 (36.6%) had high- and 199/314 (63.4%) patients had low-grade tumors. Patients with high-grade tumors showed higher preoperative calcitonin levels compared with low-grade (542 vs. 76 pg/mL, <i>p</i> < 0.01) as well as larger tumor size (2.3 vs. 1.1 cm, <i>p</i> < 0.01) and more frequent multifocality (22.6 vs. 12.1%, <i>p</i> = 0.01), minimal extrathyroidal extension (30.4 vs. 9.5%, <i>p</i> < 0.01), and lymph node metastases (63.5 vs. 27.6%, <i>p</i> < 0.01). Overall, patients with high-grade showed lower DSS, LRFS, and DMFS (<i>p</i> < 0.01). Grouping the whole cohort according to different disease extent at diagnosis, only in the case of localized disease, patients with high-grade tumors had significantly lower LRFS compared with low-grade. Similarly, in the other subgroups, we did not identify any difference in DSS, LRFS, and DMFS. Moreover, in the case of <i>RET</i> aggressive mutations, no differences in DSS, LRFS, and DMFS were observed between high- and low-grade tumors. <b><i>Conclusions:</i></b> We confirmed the usefulness of IMTCGS in predicting DSS, LRFS, and DMFS. However, it finds the best utility in patients with a lower risk of recurrence and mortality, identifying those rare cases with more aggressive clinical behavior. Conversely, when laterocervical lymph nodes (N1), distant metastasis (M1), or <i>RET</i> mutations, particularly <i>M918T</i> or <i>indels</i>, are already present at diagnosis, the role of IMTCGS in predicting DSS, DMFS, and LRFS becomes less relevant.</p>","PeriodicalId":23016,"journal":{"name":"Thyroid","volume":" ","pages":"387-396"},"PeriodicalIF":6.7000,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"The Usefulness of the International Grading System in the Management of Sporadic Medullary Thyroid Carcinoma.\",\"authors\":\"Alessandro Prete, Liborio Torregrossa, Carla Gambale, Raffaele Ciampi, Teresa Ramone, Cristina Romei, Virginia Cappagli, Paolo Piaggi, Clara Ugolini, Gabriele Materazzi, Rossella Elisei, Antonio Matrone\",\"doi\":\"10.1089/thy.2024.0444\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p><b><i>Background:</i></b> The international medullary thyroid carcinoma (MTC) grading system (IMTCGS) has been proposed as an independent tool to predict disease-specific survival (DSS), distant metastasis-free survival (DMFS), and locoregional recurrence-free survival (LRFS). 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引用次数: 0
摘要
背景:国际甲状腺髓样癌(MTC)分级系统(IMTCGS)已被提出作为预测疾病特异性生存(DSS)、远端无转移生存(DMFS)和局部无复发生存(LRFS)的独立工具。我们的目的是评估IMTCGS在散发性MTCs中的表现,并将其与传统预后因素的预测能力进行比较。方法:在一项回顾性队列研究中,我们评估了来自比萨大学医院的314例散发性MTC患者的数据。我们根据诊断时疾病的程度将患者分为局限性(183/314,58.3%)(局限于甲状腺)、区域性(100/314,31.8%)(局限于颈部,累及甲状腺周围组织和/或区域淋巴结)和远处(31/314,9.9%)(远处转移)疾病。314例患者中有212例(67.5%)存在体细胞突变。病理专家区分高、低级别肿瘤。结果:根据IMTCGS, 115/314(36.6%)患者为高级别肿瘤,199/314(63.4%)患者为低级别肿瘤。与低级别肿瘤患者相比,高级别肿瘤患者术前降钙素水平较高(542 vs. 76 pg/mL, p < 0.01),肿瘤体积较大(2.3 vs. 1.1 cm, p < 0.01),多发多发多发灶(22.6 vs. 12.1%, p = 0.01),甲状腺外扩展极小(30.4 vs. 9.5%, p < 0.01),淋巴结转移(63.5 vs. 27.6%, p < 0.01)。总体而言,高级别患者的DSS、LRFS和DMFS均较低(p < 0.01)。根据诊断时不同的病变程度对整个队列进行分组,只有在局部病变的情况下,高级别肿瘤患者的LRFS明显低于低级别肿瘤患者。同样,在其他亚组中,我们没有发现DSS、LRFS和DMFS的任何差异。此外,在RET侵袭性突变的情况下,高级别和低级别肿瘤之间的DSS、LRFS和DMFS没有差异。结论:我们证实了IMTCGS在预测DSS、LRFS和DMFS方面的有效性。然而,它发现在复发和死亡风险较低的患者中最有效,识别那些罕见的具有更积极临床行为的病例。相反,当颈侧淋巴结(N1)、远处转移(M1)或RET突变(特别是M918T或indels)在诊断时已经存在时,IMTCGS在预测DSS、DMFS和LRFS中的作用就变得不那么相关了。
The Usefulness of the International Grading System in the Management of Sporadic Medullary Thyroid Carcinoma.
Background: The international medullary thyroid carcinoma (MTC) grading system (IMTCGS) has been proposed as an independent tool to predict disease-specific survival (DSS), distant metastasis-free survival (DMFS), and locoregional recurrence-free survival (LRFS). We aimed to evaluate the performance of IMTCGS in our series of sporadic MTCs and to compare its predictive power with conventional prognostic factors. Methods: In a retrospective cohort study, we evaluated data from 314 patients with sporadic MTC, all managed at the Pisa University Hospital. We divided patients according to the extent of the disease at diagnosis into localized (183/314, 58.3%) (confined to the thyroid), regional (100/314, 31.8%) (limited to the neck, involving surrounding thyroid tissues and/or regional lymph nodes), and distant (31/314, 9.9%) (distant metastases) disease. Data about somatic mutations were available in 212/314 (67.5%) patients. Expert pathologists differentiated high- and low-grade tumors. Results: According to the IMTCGS, 115/314 (36.6%) had high- and 199/314 (63.4%) patients had low-grade tumors. Patients with high-grade tumors showed higher preoperative calcitonin levels compared with low-grade (542 vs. 76 pg/mL, p < 0.01) as well as larger tumor size (2.3 vs. 1.1 cm, p < 0.01) and more frequent multifocality (22.6 vs. 12.1%, p = 0.01), minimal extrathyroidal extension (30.4 vs. 9.5%, p < 0.01), and lymph node metastases (63.5 vs. 27.6%, p < 0.01). Overall, patients with high-grade showed lower DSS, LRFS, and DMFS (p < 0.01). Grouping the whole cohort according to different disease extent at diagnosis, only in the case of localized disease, patients with high-grade tumors had significantly lower LRFS compared with low-grade. Similarly, in the other subgroups, we did not identify any difference in DSS, LRFS, and DMFS. Moreover, in the case of RET aggressive mutations, no differences in DSS, LRFS, and DMFS were observed between high- and low-grade tumors. Conclusions: We confirmed the usefulness of IMTCGS in predicting DSS, LRFS, and DMFS. However, it finds the best utility in patients with a lower risk of recurrence and mortality, identifying those rare cases with more aggressive clinical behavior. Conversely, when laterocervical lymph nodes (N1), distant metastasis (M1), or RET mutations, particularly M918T or indels, are already present at diagnosis, the role of IMTCGS in predicting DSS, DMFS, and LRFS becomes less relevant.
期刊介绍:
This authoritative journal program, including the monthly flagship journal Thyroid, Clinical Thyroidology® (monthly), and VideoEndocrinology™ (quarterly), delivers in-depth coverage on topics from clinical application and primary care, to the latest advances in diagnostic imaging and surgical techniques and technologies, designed to optimize patient care and outcomes.
Thyroid is the leading, peer-reviewed resource for original articles, patient-focused reports, and translational research on thyroid cancer and all thyroid related diseases. The Journal delivers the latest findings on topics from primary care to clinical application, and is the exclusive source for the authoritative and updated American Thyroid Association (ATA) Guidelines for Managing Thyroid Disease.