Chow S M, Chan J K C, Tiu S C, Choi K L, Tang D L C, Law S C K
Department of Clinical Oncology, Queen Elizabeth Hospital, 30 Gascoigne Road, Hong Kong.
Hong Kong Med J. 2005 Aug;11(4):251-8.
To study the clinical parameters and treatment outcome of medullary thyroid carcinoma in Hong Kong Chinese patients.
Retrospective study.
Regional oncology unit, Hong Kong.
Patients with medullary thyroid carcinoma who were identified among 1656 patients with thyroid malignancies seen in a single institute in Hong Kong from January 1960 to June 2003.
Ten-year cause-specific survival, locoregional failure-free survival, and distant metastasis failure-free survival.
Twenty-two (1.3%) patients with medullary thyroid carcinoma were identified. The mean age at diagnosis was 43.7 (standard deviation, 16.5) years. The sex ratio was 1:1. The 10-year cause-specific survival, locoregional failure-free survival, and distant metastasis failure-free survival were 75.4%, 82.0%, and 62.4%, respectively. Lymph node metastasis was present in seven (31.8%) patients at diagnosis. Distant metastasis developed in nine (40.9%) patients: lung, 3 (13.6%); bone, 5 (22.7%); liver, 2 (9.1%); mediastinum, 4 (18.2%). Seven (31.8%) patients died of distant metastasis. Mediastinal (n=3) and bone metastases (n=3) were important causes of death. Genetic study confirmed multiple endocrine neoplasia type 2A in 3 (25.0%) of 12 patients who all had bilateral and multifocal diseases. Younger age (<45 years) was associated with better survival, better locoregional control, and less distant metastasis. Patients with pT1N0 disease (n=3) had an excellent prognosis: all were disease-free following total thyroidectomy. Among eight patients who received external radiation therapy, seven achieved good locoregional control. In seven patients with lymph node metastasis, external radiation therapy gave 100% (4/4) locoregional control compared with 33.3% (1/3) in those without external radiation therapy. Chemotherapy using dacarbazine and 5-fluorouracil was tried in three patients with poor response.
Early stage (T1N0) medullary thyroid carcinoma is associated with a very good prognosis. Postoperative external radiation therapy can achieve good locoregional control in patients with lymph node metastasis or locally advanced disease.
研究香港华裔甲状腺髓样癌患者的临床参数及治疗结果。
回顾性研究。
香港地区肿瘤中心。
1960年1月至2003年6月在香港一家机构就诊的1656例甲状腺恶性肿瘤患者中确诊的甲状腺髓样癌患者。
10年病因特异性生存率、局部区域无复发生存率和远处转移无复发生存率。
确诊22例（1.3%）甲状腺髓样癌患者。诊断时的平均年龄为43.7（标准差16.5）岁。男女比例为1:1。10年病因特异性生存率、局部区域无复发生存率和远处转移无复发生存率分别为75.4%、82.0%和62.4%。诊断时有7例（31.8%）患者出现淋巴结转移。9例（40.9%）患者发生远处转移：肺转移3例（13.6%）；骨转移5例（22.7%）；肝转移2例（9.1%）；纵隔转移4例（18.2%）。7例（31.8%）患者死于远处转移。纵隔转移（n = 3）和骨转移（n = 3）是重要的死亡原因。基因研究证实，12例双侧多灶性疾病患者中有3例（25.0%）患有2A型多发性内分泌腺瘤病。年龄较小（<45岁）与更好的生存率、更好的局部区域控制和更少的远处转移相关。pT1N0期疾病患者（n = 3）预后极佳：全甲状腺切除术后均无疾病复发。8例接受外照射治疗的患者中，7例获得了良好的局部区域控制。7例有淋巴结转移的患者中，外照射治疗的局部区域控制率为100%（4/4），而未接受外照射治疗的患者为33.3%（1/3）。3例患者尝试使用达卡巴嗪和5-氟尿嘧啶化疗，效果不佳。
早期（T1N0）甲状腺髓样癌预后极佳。术后外照射治疗可使有淋巴结转移或局部晚期疾病的患者获得良好的局部区域控制。
