日本遗传性甲状腺髓样癌的现状

Current status of inherited medullary thyroid carcinoma in Japan.

作者信息

Takami H, Hosoda Y

机构信息

KEIO UNIV,SCH MED,DEPT PATHOL,TOKYO 160,JAPAN.

出版信息

Oncol Rep. 1996 Sep;3(5):943-6. doi: 10.3892/or.3.5.943.

DOI:10.3892/or.3.5.943

PMID:21594487

Abstract

We conducted a large-scale nationwide questionnaire survey to characterize the clinical course of inherited medullary thyroid carcinoma (MTC). Out of a total of 634 MTC patients (M:F ratio 1:2.5), 175 patients had multiple endocrine neoplasia (MEN) 2A, 49 had familial non-MEN medullary thyroid carcinoma (FMTC), 20 had MEN 2B, and 390 had sporadic MTC. Median age at the time of diagnosis was 40.3, 43.0, 26.5 and 48.5 years, respectively. Total thyroidectomy plus modified neck dissection (MND) was the most common operation. The 5-year survival rates were 96.9% in MEN 2A, 100% in FMTC, 73.8% in MEN 2B, 90.8% in sporadic MTC. The outcome in order from best to worst was: FMTC, MEN 2A, sporadic MTC, and MEN 2B, with MEN 2B considered the most aggressive type.

摘要

我们开展了一项全国范围的大规模问卷调查，以描述遗传性甲状腺髓样癌（MTC）的临床病程。在总共634例MTC患者中（男女比例为1:2.5），175例患有多发性内分泌腺瘤病（MEN）2A，49例患有家族性非MEN甲状腺髓样癌（FMTC），20例患有MEN 2B，390例患有散发性MTC。诊断时的中位年龄分别为40.3岁、43.0岁、26.5岁和48.5岁。全甲状腺切除术加改良颈部淋巴结清扫术（MND）是最常见的手术方式。MEN 2A的5年生存率为96.9%，FMTC为100%，MEN 2B为73.8%，散发性MTC为90.8%。预后从最好到最差依次为：FMTC、MEN 2A、散发性MTC和MEN 2B，其中MEN 2B被认为是最具侵袭性的类型。