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Regenerative Medicine

Notable response to nivolumab during the treatment of SMARCA4-deficient thoracic sarcoma: a case report

    Yuki Iijima

    The Department of Respiratory Medicine, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    ,
    Rie Sakakibara

    *Author for correspondence: Tel.: +81 358 035 954; Fax: +81 358 030 260;

    E-mail Address: rsakakibara.pulm@tmd.ac.jp

    The Department of Respiratory Medicine, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    ,
    Masahiro Ishizuka

    The Department of Respiratory Medicine, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    ,
    Takayuki Honda

    The Department of Respiratory Medicine, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    ,
    Tsuyoshi Shirai

    The Department of Respiratory Medicine, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    ,
    Tsukasa Okamoto

    The Department of Respiratory Medicine, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    ,
    Tomoya Tateishi

    The Department of Respiratory Medicine, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    ,
    Hiroyuki Sakashita

    The Department of Respiratory Medicine, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    Cancer Center, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    ,
    Meiyo Tamaoka

    The Department of Respiratory Medicine, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    ,
    Akira Takemoto

    The Department of Pathology, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    ,
    Yuichi Kumaki

    Cancer Center, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    ,
    Sadakatsu Ikeda

    Cancer Center, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    &
    Yasunari Miyazaki

    Cancer Center, Tokyo Medical & Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan

    Published Online:https://doi.org/10.2217/imt-2019-0142

    SMARCA4-deficient thoracic sarcoma is a rare tumor typically presenting as a mediastinal mass. The prognosis is estimated to be poor, and no effective treatment has been established. We present a case of a 76-year-old man who was diagnosed with SMARCA4-deficient thoracic sarcoma. The provisional diagnosis was carcinoma of unknown primary but subsequently corrected to SMARCA4-deficient thoracic sarcoma based on the panel-based cancer gene screening and immunohistochemistry. Cytotoxic chemotherapy as the first- and second-line did not reveal enough therapeutic effects but third-line therapy using nivolumab showed marked tumor regression, which was sustained. This is the first case report of SMARCA4-deficient thoracic sarcoma showing a good response to nivolumab. Immune checkpoint inhibitor might be therapeutic candidates for this type of tumor.

    Papers of special note have been highlighted as: • of interest.

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