Tsukiji Jun, Kaneko Takeshi, Saito Haruhiro, Kobayashi Masayoshi, Kakemizu Nobumasa, Komatsu Shigeru, Nishiyama Harumi, Amano Hiroko, Miura Kenji, Satoh Takashi, Shinohara Takeshi, Mishima Wataru, Yamakawa Yasushi, Nishihira Ryuuichi, Miyashita Akira, Noda Kazumasa, Ishigatsubo Yoshiaki, Matsuse Takeshi
Nihon Kokyuki Gakkai Zasshi. 2004 Sep;42(9):820-4.
We encountered a very rare case of cT0N2M0 small cell lung cancer (SCLC) with Lambert-Eaton myasthenic syndrome (LEMS). A 69-year-old man with a complaint of muscle weakness was admitted to our hospital. Although his chest radiograph on admission showed no abnormal findings, CT scanning detected a mediastinal lymphadenopathy. Also, 2-[18F]-2-fluorodeoxy-D-glucose position emission tomography (FDG-PET) revealed increased accumulation in the same portion in the mediastinum. A diagnosis of LEMS was made from the distinctive electromyogram (EMG) findings (waning and waxing phenomenon in response to low-and high-frequency repetitive stimulation, respectively) in combination with the increased serum level of a P/Q-type anti-voltage-gated calcium channel (VGCC) antibody. Subsequent histopathological diagnosis by mediastinoscopic resection of a paraaortic lymph node was small cell carcinoma. No distant metastasis was detected by MRI of the brain, abdominal CT scan or an FDG-PET. Eight courses of chemotherapy (carboplatin + etoposide) with radiotherapy of the mediastinum (for a total dose of 45 Gy) was performed. A decreased serum level of P/Q-type anti-VGCC antibody titers followed by marked improvement of neurological dysfunction (muscle weakness, gait disturbance and scanning speech) and of an EMG finding (a loss of waning phenomenon) was observed. A close relationship between reduction of the antibody titers and improvement of neurological symptoms after the therapy was noticed. It was suggested that monitoring the level of a P/Q-type anti-VGCC antibody titer in the serum is important for evaluating the efficacy of chemotherapy for LEMS associated with SCLC.
我们遇到了一例非常罕见的cT0N2M0小细胞肺癌(SCLC)合并兰伯特-伊顿肌无力综合征(LEMS)的病例。一名69岁男性因肌肉无力主诉入院。尽管入院时胸部X线片未见异常,但CT扫描发现纵隔淋巴结肿大。此外,2-[18F]-2-氟脱氧-D-葡萄糖正电子发射断层扫描(FDG-PET)显示纵隔同一部位放射性摄取增加。根据独特的肌电图(EMG)表现(分别对低频和高频重复刺激出现波幅递减和递增现象)以及血清P/Q型抗电压门控钙通道(VGCC)抗体水平升高,诊断为LEMS。随后通过纵隔镜切除主动脉旁淋巴结进行组织病理学诊断为小细胞癌。脑部MRI、腹部CT扫描或FDG-PET均未发现远处转移。进行了8个周期的化疗(卡铂+依托泊苷)并联合纵隔放疗(总剂量45 Gy)。观察到血清P/Q型抗VGCC抗体滴度降低,随后神经功能障碍(肌肉无力、步态障碍和吟诗样言语)及EMG表现(波幅递减现象消失)明显改善。注意到治疗后抗体滴度降低与神经症状改善之间存在密切关系。提示监测血清中P/Q型抗VGCC抗体滴度水平对于评估与SCLC相关的LEMS化疗疗效具有重要意义。