Siironen Päivi, Hagström Jaana, Mäenpää Hanna O, Louhimo Johanna, Arola Johanna, Haglund Caj
a Department of Surgery , University of Helsinki and Helsinki University Hospital , Helsinki , Finland .
b Department of Pathology , Haartman Institute and HUSLab, University of Helsinki and Helsinki University Hospital , Helsinki , Finland .
Acta Oncol. 2016;55(3):357-64. doi: 10.3109/0284186X.2015.1070963. Epub 2015 Sep 4.
Background Total thyroidectomy is the treatment of choice for medullary thyroid carcinoma (MTC), but the extent of neck dissection is controversial. Lymph node metastases, distant metastases, and old age are known predictors of poor survival. Patients Patients treated for primary MTC at Helsinki University Hospital from 1990 to 2009 were included (n = 54). Their clinical characteristics, treatment, and outcome were analysed retrospectively, these patients were followed until death or their last follow-up date. Results At last follow-up (3.4-23 years), of 54 MTC patients, 19 (35%) were disease-free, 17 (32%) were alive with disease, and 12 (22%) had died of MTC; six patients died of unrelated causes (11%). All disease-free patients were node negative and had normal postoperative calcitonin level. Of 19 disease-free patients, only four (21%) had undergone lymph node dissection. All patients who died of MTC were Stage IV at diagnosis and died with distant metastases. Disease-specific five-and 10-year survival was 84% and 76.2%. Advanced T-stage (p = 0.004), lymph node metastases (p < 0.001), distant metastases (p < 0.001), stage (p < 0.001), and elevated postoperative calcitonin (p < 0.001) significantly associated with survival. Conclusions Lymph node metastasis and elevated postoperative calcitonin are important prognostic factors. Patients with lymph node metastasis and/or elevated postoperative calcitonin with present treatments cannot become disease-free, but most of them can live a long life with metastasis.
背景 全甲状腺切除术是甲状腺髓样癌（MTC）的首选治疗方法，但颈部清扫的范围存在争议。淋巴结转移、远处转移和高龄是已知的生存不良预测因素。
患者 纳入1990年至2009年在赫尔辛基大学医院接受原发性MTC治疗的患者（n = 54）。对他们的临床特征、治疗和结局进行回顾性分析，这些患者随访至死亡或最后随访日期。
结果 在最后随访时（3.4 - 23年），54例MTC患者中，19例（35%）无疾病，17例（32%）带瘤生存，12例（22%）死于MTC；6例患者死于无关原因（11%）。所有无疾病患者均无淋巴结转移且术后降钙素水平正常。19例无疾病患者中，仅4例（21%）接受了淋巴结清扫。所有死于MTC的患者诊断时均为IV期且死于远处转移。疾病特异性5年和10年生存率分别为84%和76.2%。进展期T分期（p = 0.004）、淋巴结转移（p < 0.001）、远处转移（p < 0.001）、分期（p < 0.001）和术后降钙素升高（p < 0.001）与生存显著相关。
结论 淋巴结转移和术后降钙素升高是重要的预后因素。目前治疗下有淋巴结转移和/或术后降钙素升高的患者无法实现无疾病状态，但他们中的大多数人虽有转移仍可长期生存。
