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初始治疗后生化未完全缓解的散发性甲状腺髓样癌的临床演变。

Clinical Evolution of Sporadic Medullary Thyroid Carcinoma With Biochemical Incomplete Response After Initial Treatment.

机构信息

Department of Clinical and Experimental Medicine, Unit of Endocrinology, Pisa University Hospital, 56124, Pisa, Italy.

Department of Surgical, Medical, Molecular Pathology and Critical Area, Anatomic Pathology Section, University Hospital of Pisa, 56126, Pisa, Italy.

出版信息

J Clin Endocrinol Metab. 2023 Jul 14;108(8):e613-e622. doi: 10.1210/clinem/dgad061.

Abstract

CONTEXT

The clinical response after surgery is a determinant in the management of patients with medullary thyroid carcinoma (MTC). In case of excellent or structural incomplete response, the follow-up strategies are well designed. Conversely, in case of biochemical incomplete response (BiR) the management is not clearly defined.

OBJECTIVE

This work aimed to evaluate the overall and per-site prevalence of structural disease detection in sporadic MTC patients with BiR and to assess the predictive value of various clinical, biochemical, and genetic features.

METHODS

We evaluated data of 599 consecutive patients surgically treated for sporadic MTC (2000-2018) and followed-up at the endocrine unit of the University Hospital of Pisa.

RESULTS

After a median of 5 months from surgery, 145 of 599 (24.2%) patients were classified as BiR. Structural disease was detected in 64 of 145 (44.1%), after a median time of 3.3 years. In 73.6%, structural disease was detected at a single site, prevalently cervical lymph nodes. Among several others, at the time of first evaluation after surgery, only basal calcitonin (bCTN) and stage IVa/b were independent predictive factors. Also, structural disease was more frequent in patients with shorter CTN doubling time and somatic RET mutation.

CONCLUSION

In sporadic MTC patients with BiR, the risk of detection of structural disease was about 50% at 10 years. Higher bCTN levels and staging predicted the risk of detecting structural disease. According to these findings, stricter follow-up should be reserved for MTC with BiR and elevated values of bCTN and to those with an advanced stage. Long follow-up should be considered for all BiR patients since 50% of them develop structural disease within 10 years.

摘要

背景

手术后的临床反应是决定甲状腺髓样癌(MTC)患者治疗的一个决定因素。在完全缓解或结构不完全缓解的情况下,随访策略设计得很好。相反,在生化不完全缓解(BiR)的情况下,管理尚不清楚。

目的

本研究旨在评估伴有 BiR 的散发性 MTC 患者结构疾病的总体和各部位检出率,并评估各种临床、生化和遗传特征的预测价值。

方法

我们评估了 599 例连续接受手术治疗的散发性 MTC 患者(2000-2018 年)的数据,并在比萨大学医院的内分泌科进行了随访。

结果

手术后中位数 5 个月时,599 例患者中有 145 例(24.2%)被归类为 BiR。中位数时间 3.3 年后,在 145 例中有 64 例(44.1%)检测到结构疾病。在 73.6%的病例中,结构疾病仅在单一部位发现,主要为颈部淋巴结。在其他多个因素中,仅术后首次评估时的基础降钙素(bCTN)和 IVa/b 期是独立的预测因素。此外,在 bCTN 倍增时间较短和存在体细胞 RET 突变的患者中,结构疾病更常见。

结论

在伴有 BiR 的散发性 MTC 患者中,10 年内检测到结构疾病的风险约为 50%。较高的 bCTN 水平和分期预测了检测结构疾病的风险。根据这些发现,应保留更严格的随访,用于 bCTN 值升高和分期较高的伴有 BiR 的 MTC 患者,也应保留用于所有伴有 BiR 的患者,因为其中 50%的患者在 10 年内会出现结构疾病。

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