Pelizzo M R, Boschin I M, Bernante P, Toniato A, Piotto A, Pagetta C, Nibale O, Rampin L, Muzzio P C, Rubello D
Department of Medical and Surgical Sciences, Institute of Surgical Pathology, University of Padova, Padova, Italy.
Eur J Surg Oncol. 2007 May;33(4):493-7. doi: 10.1016/j.ejso.2006.10.021. Epub 2006 Nov 27.
The analysis of a 37-year retrospective study on diagnosis, prognostic variables, treatment and outcome of a large group of medullary thyroid cancer (MTC) patients was conducted, in order to plan a possible evidence-based management process.
Between Jan 1967 to Dec 2004, 157 consecutive MTC patients underwent surgery in our centre: 60 males and 97 females, mean age 47.3 years (range 6-79). Total thyroidectomy was performed in 143 patients (91.1%); central compartment (CC) node dissection (level VI) in 41 patients; central plus lateral compartment (LC) node dissection (levels II, III, and IV) in 82 patients. Subtotal thyroidectomy was initially performed in 14 cases: 10 of them were re-operated because of persistence of elevated serum calcitonin levels.
After a median post-surgical follow-up of 68 months (range 2-440 months), 42.9% of patients were living disease-free, 39.8% were living with disease, 3.1% were deceased due to causes different from MTC, and 3.2% were deceased due to MTC. The overall 10-year survival rate was 72%. At uni-variate statistical analysis (a) patient's age at initial treatment (>45 years; >/=45 years), (b) sporadic vs. hereditary MTC, (c) disease stage, and (d) the extent of surgical approach resulted as significant variables. Instead, at multivariate statistical analysis, only (a) patient's age at initial diagnosis, (b) disease stage, and (c) the extent of surgery resulted as significant and independent prognostic variables influencing survival.
The presence of lymph node and distant metastases at first diagnosis significantly worsened prognosis and survival rate in our series. Early diagnosis of MTC is very important, allowing complete surgical cure in Stages I and II patients. Due to the relatively bad prognosis of MTC, especially for disease Stages III and IV, it appears reasonable to recommend radical surgery including total thyroidectomy plus CC lymphoadenectomy as the treatment of choice, plus LC lymphoadenectomy in patients with palpable and/or ultrasound enlarged neck lymph nodes.
对一大组甲状腺髓样癌（MTC）患者的诊断、预后变量、治疗及结果进行37年的回顾性研究分析，以规划一个可能基于证据的管理流程。
1967年1月至2004年12月期间，157例连续性MTC患者在本中心接受手术：男性60例，女性97例，平均年龄47.3岁（范围6 - 79岁）。143例患者（91.1%）接受了全甲状腺切除术；41例患者进行了中央区（CC）淋巴结清扫（Ⅵ区）；82例患者进行了中央区加侧方区（LC）淋巴结清扫（Ⅱ、Ⅲ和Ⅳ区）。最初14例患者进行了次全甲状腺切除术：其中10例因血清降钙素水平持续升高而再次手术。
术后中位随访68个月（范围2 - 440个月），42.9%的患者无病生存，39.8%的患者带病生存，3.1%的患者因非MTC原因死亡，3.2%的患者因MTC死亡。总体10年生存率为72%。单因素统计分析显示（a）初始治疗时患者年龄（>45岁；≥45岁）、（b）散发性与遗传性MTC、（c）疾病分期以及（d）手术方式的范围是显著变量。相反，多因素统计分析显示，只有（a）初始诊断时患者年龄、（b）疾病分期以及（c）手术范围是影响生存的显著且独立的预后变量。
在我们的系列研究中，首次诊断时存在淋巴结和远处转移显著恶化了预后和生存率。MTC的早期诊断非常重要，可使Ⅰ期和Ⅱ期患者获得完全手术治愈。由于MTC预后相对较差，尤其是Ⅲ期和Ⅳ期疾病，推荐根治性手术，包括全甲状腺切除术加CC区淋巴结清扫作为首选治疗方法，对于可触及和/或超声显示颈部淋巴结肿大的患者，加做LC区淋巴结清扫似乎是合理的。
