Yamano Tomoki, Hamanaka Michiko, Babaya Akihito, Kimura Kei, Kobayashi Masayoshi, Fukumoto Miki, Tsukamoto Kiyoshi, Noda Masafumi, Matsubara Nagahide, Tomita Naohiro, Sugihara Kenichi
Division of Lower Gastrointestinal Surgery, Department of Surgery, Hyogo College of Medicine, Nishinomiya, Japan.
Tokyo Medical and Dental University, Tokyo, Japan.
Cancer Sci. 2017 Feb;108(2):243-249. doi: 10.1111/cas.13123.
Lynch syndrome (LS) and familial adenomatous polyposis (FAP) are major sources of hereditary colorectal cancer (CRC) and are associated with other malignancies. There is some heterogeneity in management strategies in Japan. We undertook a survey of management of hereditary CRC in hospitals that are members of the Japan Society of Colorectal Cancer Research. One hundred and ninety departments responded, of which 127 were from designated cancer care hospitals (DCCHs) according to the Japanese government. There were 25 488 operations for CRC in these departments in 2015. The DCCHs performed better with regard to usage of Japan Society of Colorectal Cancer Research guidelines, referring new CRC patients for LS screening, and having in-house genetic counselors and knowledge of treatment for LS. There were 174 patients diagnosed with LS and 602 undergoing follow-up in 2011-2015, which is fewer than the number expected from CRC operations in 2015. These numbers were not affected by whether the institution was a DCCH. Universal screening for LS was carried out in 8% of the departments. In contrast, 541 patients were diagnosed with FAP and 273 received preventive proctocolectomy/colectomy in 2011-2015. The DCCH departments undertook more surgery than non-DCCH departments, although most of the management, including surgical procedures and use of non-steroidal anti-inflammatory drugs, was similar. Management of desmoid tumor in the abdominal cavity differed according to the number of patients treated. In conclusion, there was heterogeneity in management of LS but not FAP. Most patients with LS may be overlooked and universal screening for LS is not common in Japan.
林奇综合征（LS）和家族性腺瘤性息肉病（FAP）是遗传性结直肠癌（CRC）的主要病因，且与其他恶性肿瘤相关。在日本，管理策略存在一定的异质性。我们对日本结直肠癌研究学会成员医院的遗传性CRC管理情况进行了一项调查。190个科室做出了回应，其中127个来自日本政府指定的癌症护理医院（DCCHs）。2015年，这些科室共进行了25488例CRC手术。DCCHs在使用日本结直肠癌研究学会指南、将新的CRC患者转诊进行LS筛查、配备内部遗传咨询师以及掌握LS治疗知识方面表现更佳。2011 - 2015年期间，有174例患者被诊断为LS，602例正在接受随访，这一数字低于根据2015年CRC手术预期的数量。这些数字不受该机构是否为DCCH的影响。8%的科室对LS进行了普遍筛查。相比之下，2011 - 2015年期间，有541例患者被诊断为FAP，273例接受了预防性直肠结肠切除术/结肠切除术。DCCH科室进行的手术比非DCCH科室更多，不过包括手术程序和非甾体抗炎药的使用在内的大多数管理措施是相似的。腹腔内硬纤维瘤的管理因治疗患者数量而异。总之，LS的管理存在异质性，但FAP并非如此。大多数LS患者可能被忽视，且在日本对LS进行普遍筛查并不常见。
