Endocrine Unit, Department of Medical Therapeutics, Alexandra Hospital, Athens University School of Medicine, 80 Vass Sofias Avenue, 11528 Athens, Greece.
Eur J Endocrinol. 2012 Dec;167(6):799-808. doi: 10.1530/EJE-12-0388. Epub 2012 Sep 18.
Medullary thyroid carcinoma (MTC) has varying clinical course. We assessed trends in MTC presentation during the last 34 years.
Retrospective study.
One hundred and fifty one patients (44.4% males) were followed for 0.934 years. Patients were classified according to year of diagnosis: group 1, 1977-2000 (n=53) and group 2, 2001-2011 (n=98). Extent of disease at diagnosis, during follow-up, number of surgeries, and pre- and postoperative calcitonin levels were recorded.
In total, 48.34% reported family history of MTC. Group 1 had larger tumors (median 1.70 (intraquartile range (IQR) 1.7) vs 1.1 (1.2) cm, P=0.045, Mann-Whitney), they presented less frequently micro-MTCs (27.8 vs 46.1%, P=0.045), and underwent more multiple surgeries (63.3 vs 20.0%, P<0.001). Group 1 had more frequently progressive disease (35.8 vs 12.2%, P=0.003) and distant metastasis at follow-up (39.7 vs 17.4%, P=0.017). Chronological group (HR 0.15, 95% CI 0.03-0.68, P=0.015) and distant metastases at follow-up (HR 0.07, 95% CI 0.015-0.30, P=0.001) were independently associated with 10-year disease progression (P<0.001). In sporadic cases, cervical lymph node invasion and distant metastases at diagnosis were more frequent in group 1 (72.7 vs 45.5%, P=0.032 and 27.3 vs 5%, P=0.019 respectively); disease stage at diagnosis was more advanced (P=0.004). They underwent more multiple surgeries (P<0.001), presented more frequently distant metastasis at follow-up (67.7 vs 20.0%, P=0.002), had less frequently remission, and more frequently progressive disease (21.4 vs 58.0% and 64.3 vs 14.0% respectively, P<0.001). Postoperative calcitonin levels were higher (P=0.024).
Recently, an increase in micro-MTCs is observed, while indices of invasiveness and persistence of disease are better. Increased awareness in familial cases, routine calcitonin measurements, and improved surgical procedures could be responsible.
甲状腺髓样癌(MTC)的临床表现各异。本研究旨在评估过去 34 年来 MTC 患者的临床表现变化趋势。
对 151 例患者(44.4%为男性)进行了 0.934 年的随访。根据诊断年份将患者分为两组:1977-2000 年组(n=53)和 2001-2011 年组(n=98)。记录患者的诊断时疾病严重程度、随访期间的疾病进展情况、手术次数以及术前和术后降钙素水平。
共有 48.34%的患者有 MTC 家族史。与组 1 相比,组 2 的肿瘤更小(中位数 1.70(四分位距(IQR)1.7)vs 1.1(1.2)cm,P=0.045,Mann-Whitney),微髓样癌的发生率更低(27.8% vs 46.1%,P=0.045),手术次数更多(63.3% vs 20.0%,P<0.001)。组 1 更易发生疾病进展(35.8% vs 12.2%,P=0.003)和随访时出现远处转移(39.7% vs 17.4%,P=0.017)。时间序列分组(HR 0.15,95%CI 0.03-0.68,P=0.015)和随访时出现远处转移(HR 0.07,95%CI 0.015-0.30,P=0.001)是疾病进展 10 年的独立预测因素(P<0.001)。在散发型病例中,组 1 患者的颈部淋巴结侵犯和诊断时远处转移的发生率更高(72.7% vs 45.5%,P=0.032 和 27.3% vs 5%,P=0.019);疾病分期更晚(P=0.004)。他们接受了更多次的手术(P<0.001),随访时更易出现远处转移(67.7% vs 20.0%,P=0.002),缓解率更低,疾病进展率更高(21.4% vs 58.0%和 64.3% vs 14.0%,P<0.001)。术后降钙素水平更高(P=0.024)。
近期,微髓样癌的发病率增加,而侵袭性和疾病持续存在的指标则更好。这可能与家族性病例的认识提高、常规降钙素检测以及手术技术的改进有关。