Jiang Jing, Huang Ting, Lin Xianlei, Zhang Yu, Yang Xuefei, Huang Ling, Ye Zhifeng, Ren Xingchang, Teng Lisong, Li Jun, Kong Mei, Lian Liyan, Lu Jinhua, Zhong Yazhen, Lin Zechen, Xu Ming, Chen Yin, Lin Shengyou
Department of Oncology, Hangzhou Traditional Chinese Medicine (TCM) Hospital Affiliated to Zhejiang Chinese Medical University, Hangzhou, China.
The Third Clinical Medical College, Zhejiang Chinese Medical University, Hangzhou, China.
Front Oncol. 2022 May 10;12:896024. doi: 10.3389/fonc.2022.896024. eCollection 2022.
With the modern technological developments in the diagnosis and treatment of cancer, the survival rate of cancer patients has increased. On the other hand, the incidence of multiple primary tumors is increasing annually. Lynch syndrome (LS), an autosomal dominant disorder with germline mutations in DNA mismatch repair genes, increases the risk of cancer in patients carrying those mutations. In this report, we present an extremely rare case of an 81-year-old male patient with eight primary malignancies and LS. The patient is still alive having survived for more than 41 years since the initial discovery of the first tumor. The eighth and most recently diagnosed primary cancer was a malignant peripheral nerve sheath tumor. Although there have been numerous reports of malignancies in LS, malignant peripheral nerve sheath tumors have not been reported previously with LS. Here, we report, to the best of our knowledge, the first case of a malignant peripheral nerve sheath tumor with LS.
随着癌症诊断和治疗方面的现代技术发展，癌症患者的生存率有所提高。另一方面，多原发性肿瘤的发病率每年都在上升。林奇综合征（LS）是一种常染色体显性疾病，其DNA错配修复基因存在种系突变，会增加携带这些突变的患者患癌风险。在本报告中，我们呈现了一例极为罕见的病例，一名81岁男性患者患有8种原发性恶性肿瘤且患有林奇综合征。自首次发现第一个肿瘤以来，该患者已存活超过41年，目前仍然健在。第八个也是最近诊断出的原发性癌症是恶性外周神经鞘瘤。尽管已有大量关于林奇综合征患者发生恶性肿瘤的报道，但此前尚未有林奇综合征合并恶性外周神经鞘瘤的报道。在此，据我们所知，我们报告了首例林奇综合征合并恶性外周神经鞘瘤的病例。
