Objective To study the clinical characteristics, the surgical treatments and the prognostic factors of medullary thyroid carcinoma. Methods Eighty-two cases of medullary thyroid carcinoma undergoing surgeries between January 1999 and December 2004 were reviewed. There were 39 males and 43 females. Age ranged from 16 to 77 years old, with a median of 46 years old. The calcitonin, chronogranin A and neuron-specific enolase were analysed by immunohistochemistry in samples,and calcitonin was detected in 24 cases. Of them, 28 cases underwent lobectomy with isthmectomy, 24 for remained lobe dissection, 16 for total thyroidectomy, and 14 only for neck and upper mediastinal lymph node dissection. Of the 82 cases, 68 cases underwent trachea esophageal ditch dissection, 53 for unilateral neck dissection, 11 for bilateral neck dissection, 13 for upper mediastinal lymph node dissecion through transcervical approach, and 5 for mediastinal lymph node dissecion through inverted T-shaped incision. Results Immunohistochemical examination revealed that the expression rates of calcitonin, chronogranin A and neuron-specific enolase were 95.8%, 88.9% and 80. 0% respectively. Total metastasis rate of neck lymph nodes was 68.8%, and the rates in level Ⅱ, Ⅲ, Ⅳ, Ⅴ andⅥ were 27.3%, 47.7%, 59.1%, 11.4% and 52.3% respectively. The overall five-year survival rate was 87.8%. The recurrent rate of contralateral lobes was 5.8% and local recurrent rate was 7.3% respectively. Univariate analysis showed that gender, age and TNM stage were significant prognostic factors. Multivariate analysis revealed that distant metastasis was an independent prognostic factor. Conclusions Standard radical surgery of the primary and metastatic lesion is key to the treatment of medullary thyroid carcinoma. Lobectomy with isthmectomy should be applied to sporadic medullary thyroid carcinoma, with regular postoperative follow-up, and total thyroidectomy to familial or bilateral medullary thyroid carcinoma. Therefore. detecting the calcitonin is very imoortant for medullary thyroid carcinoma patients' prognosis.%目的 探讨甲状腺髓样癌的临床特点及治疗方法,并对其预后及影响因素进行分析.方法 对1999年1月至2004年12月中国医学科学院肿瘤医院头颈外科收治的82例甲状腺髓样癌患者的临床资料进行回顾性分析.男性39例,女性43例;年龄16～77岁,中位年龄46岁.采用免疫组织化学方法,分别检测降钙素、嗜铬素、神经元烯醇化酶的表达水平,其中24例标本行降钙素水平检测.行患侧腺叶+峡部切除28例,甲状腺残叶切除24例,甲状腺全切除16例,单纯行颈清扫术及上纵隔淋巴清扫术14例.82例中行气管食管沟清扫68例;行单侧颈清扫53例,双侧颈清扫11例,经颈部上纵隔淋巴清扫13例,胸骨劈开上纵隔淋巴清扫5例.结果 所有病例均经病理证实为甲状腺髓样癌,免疫组织化学检查显示降钙素表达率最高,为95.8%,嗜铬素表达率为88.9%,神经元烯醇化酶表达率为80.0%.颈部淋巴转移率68.8%,Ⅱ区、Ⅲ区、Ⅳ区、Ⅴ区和Ⅵ区淋巴转移率分别为27.3%、47.7%、59.1%、11.4%和52.3%.总的5年生存率为87.8%,总的局部复发率为7.3%,对侧腺叶复发率为5.8%.单因素分析结果表明性别、年龄、TNM分期是影响预后的因素,多因素分析表明远处转移是影响预后的独立危险因素.结论 外科规范手术是甲状腺髓样癌治疗的主要方式;单侧散发型甲状腺髓样癌首次治疗时应行患侧腺叶+峡部切除,术后应密切随访,而对于双侧病灶或家族型者应行甲状腺全切除术.监测降钙素水平在甲状腺髓样癌预后有重要意义.
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