Cherian Anish Jacob, Ramakant Pooja, Pai Rekha, Manipadam Marie Therese, Elanthenral S, Chandramohan Anuradha, Hephzibah Julie, Mathew David, Naik Dhukabandhu, Paul Thomas V, Rajaratnam Simon, Thomas Nihal, Paul M J, Abraham Deepak Thomas
1Department of Endocrine Surgery, Christian Medical College and Hospital, Paul Brand building (1205), Vellore, Tamil Nadu India.
2Department of Molecular Pathology, Christian Medical College and Hospital, Vellore, India.
Indian J Surg Oncol. 2018 Mar;9(1):52-58. doi: 10.1007/s13193-017-0718-2. Epub 2017 Dec 8.
We conducted this study to evaluate the demography, clinical presentation, management and outcomes of medullary thyroid carcinoma (MTC) from the Indian context. This was a retrospective study of patients with MTC managed between January 2008 and December 2016. All pertinent data was collected and the results were analysed using STATA (v.13.1). MTC accounted for 90/2022 (4.45%) patients managed with thyroid cancer during the study period. The mean age of presentation was 40 years (range 14-70 years) with 47 males and 43 females. The most common presentation included goitre with cervical lymphadenopathy seen in 60 patients (66.7%). There were 11 patients (12.2%) with systemic metastasis at presentation. Rearranged during transfection (RET) testing was performed in 71 patients and was positive in 25 (35.2%). The mutations among these patients were seen in the following codons: 634 (12), 804 (8), 790 (3) and 618 (2). Persistent hypercalcitoninemia (calcitonin > 50 pg/ml) was observed in 62/80 (77.5%) patients. Forty patients underwent a meta-iodo-benzyl-guanidine (MIBG) scan in the postoperative period, 10 were positive. The mean duration of follow-up was 32 months and 10 patients defaulted from follow-up. Sixteen patients developed metastasis during the period of follow-up while eight patients expired. The mean survival was 85.75 months (95% CI 78.7-92.7). MTC accounted for 4.5% of thyroid carcinomas in this cohort among which 35% were hereditary. Persistent hypercalcitoninemia following surgery is seen in more than 70% of patients but this does not affect survival. RET screening should be performed for all patients with MTC as curative surgery can be offered for mutation positive offspring.
我们开展这项研究,以评估印度背景下甲状腺髓样癌(MTC)的人口统计学特征、临床表现、治疗及预后情况。这是一项对2008年1月至2016年12月期间接受治疗的MTC患者的回顾性研究。收集了所有相关数据,并使用STATA(v.13.1)进行结果分析。在研究期间,MTC占接受甲状腺癌治疗的2022例患者中的90例(4.45%)。患者的平均就诊年龄为40岁(范围14 - 70岁),其中男性47例,女性43例。最常见的表现为甲状腺肿伴颈部淋巴结肿大,60例患者(66.7%)出现此症状。11例患者(12.2%)就诊时已有全身转移。对71例患者进行了转染重排(RET)检测,其中25例(35.2%)呈阳性。这些患者的突变发生在以下密码子:634(12例)、804(8例)、790(3例)和618(2例)。80例患者中有62例(77.5%)观察到持续性降钙素血症(降钙素>50 pg/ml)。40例患者术后接受了间碘苄胍(MIBG)扫描,10例呈阳性。平均随访时间为32个月,10例患者失访。16例患者在随访期间发生转移,8例患者死亡。平均生存期为85.75个月(95%可信区间78.7 - 92.7)。在此队列中,MTC占甲状腺癌的4.5%,其中35%为遗传性。超过70%的患者术后出现持续性降钙素血症,但这并不影响生存。所有MTC患者均应进行RET筛查,因为对于突变阳性的后代可提供根治性手术。