Geller Georgia, Laskin Janessa, Cheung Winson Y, Ho Cheryl
Medical Oncology, British Columbia Cancer Agency, 600 W 10th Avenue, Vancouver, BC V5Z 4E6 Canada.
Medical Oncology, Tom Baker Cancer Centre, Calgary, AB Canada.
Thyroid Res. 2017 Sep 19;10:6. doi: 10.1186/s13044-017-0041-6. eCollection 2017.
Medullary thyroid carcinoma (MTC) accounts for 1-2% of all thyroid cancers. The clinical course of metastatic disease can be indolent. Our aim was to characterize the natural history of disease to evaluate the true proportion of patients who would be eligible for the currently available systemic therapies.
The British Columbia Cancer Agency (BCCA) provides cancer care to a population of 4.6 million. A retrospective chart review was conducted of all patients with MTC referred to the BCCA from 1991 to 2013. Clinical characteristics, pathology, treatment and outcome data were collected. Relapse free survival and overall survival was determined for patients based on staging at the time of diagnosis.
Of the 98 patients referred to the BCCA during the study period, inherited mutations were found in 6% though 60% did not undergo genetic testing. Based on clinical SEER staging at diagnosis 50% had localized disease, 38% regional, and 12% had distant metastasis. 77% had complete surgical resection of which 25% received adjuvant radiation therapy. Five year relapse free survival (RFS) for localized and regional disease was 75% and 66%, respectively ( = 0.006). Initial treatment of 23 patients with locally unresectable and metastatic disease predominantly involved multiple modalities. Of the 37 patients with relapsed or metastatic MTC only 7 (19%) patients received one or more course of chemotherapy for metastatic disease: 1 temsirolimus, 2 adriamycin, 3 sunitinib, 3 sorafenib, and 3 vandetanib. Five year OS based on clinical SEER stage: localized 93%, regional 72% and distant 33% ( < 0.001).
Localized and regional MTC treatment patterns reflect multidisciplinary management based on disease characteristics. Patients with distant disease had poor outcomes with 28% of patients dying from disease. In our cohort the minority of patients ultimately received systemic therapy due to timing and lack of TKI availability.
甲状腺髓样癌（MTC）占所有甲状腺癌的1%-2%。转移性疾病的临床病程可能较为隐匿。我们的目的是描述疾病的自然史，以评估符合目前可用全身治疗条件的患者的真实比例。
不列颠哥伦比亚癌症机构（BCCA）为460万人口提供癌症护理。对1991年至2013年转诊至BCCA的所有MTC患者进行回顾性病历审查。收集临床特征、病理、治疗和结局数据。根据诊断时的分期确定患者的无复发生存期和总生存期。
在研究期间转诊至BCCA的98例患者中，6%发现存在遗传性突变，尽管60%未接受基因检测。根据诊断时的临床SEER分期，50%为局限性疾病，38%为区域性疾病，12%有远处转移。77%接受了完整的手术切除，其中25%接受了辅助放射治疗。局限性和区域性疾病的五年无复发生存率（RFS）分别为75%和66%（P = 0.006）。23例局部不可切除和转移性疾病患者的初始治疗主要涉及多种治疗方式。在37例复发或转移性MTC患者中，只有7例（19%）患者接受了一个或多个疗程的转移性疾病化疗：1例接受替西罗莫司治疗，2例接受阿霉素治疗，3例接受舒尼替尼治疗，3例接受索拉非尼治疗，3例接受凡德他尼治疗。根据临床SEER分期的五年总生存率：局限性疾病为93%，区域性疾病为72%，远处转移为33%（P < 0.001）。
局限性和区域性MTC的治疗模式反映了基于疾病特征的多学科管理。远处疾病患者的结局较差，28%的患者死于该疾病。在我们的队列中，由于时机和缺乏酪氨酸激酶抑制剂，少数患者最终接受了全身治疗。
