Maekura Toshiya, Shimizu Shigeki, Kawaguchi Tomoya, Morimoto Masahiro, Okuma Tomohisa, Matsui Hideo, Atagi Shinji
Department of Internal Medicine, National Hospital Organization Kinki-chuo Chest Medical Center, Japan.
Intern Med. 2015;54(9):1095-8. doi: 10.2169/internalmedicine.54.3624. Epub 2015 May 1.
Synovial sarcoma (SS) commonly arises in the para-articular soft tissue; however, very few cases of intravascular SS have so far been reported. We herein describe a case of pulmonary artery SS with massive pleural effusion. A biopsy of the pleural lesions showed uniform short spindle cell proliferation, while the SYT-SSX fusion gene, which is preceded by chromosomal translocation t(X;18)(p11;q11), was detected using reverse transcription-polymerase chain reaction. Treatment with ifosfamide chemotherapy and palliative radiation therapy was effective in reducing the growth of the tumor in the pulmonary artery and pleural lesions, indicating that this regimen may be useful for the treatment of unresectable SS in the pulmonary artery.
滑膜肉瘤（SS）通常发生于关节旁软组织；然而，迄今为止，血管内滑膜肉瘤的病例报道极少。我们在此描述一例伴有大量胸腔积液的肺动脉滑膜肉瘤病例。胸膜病变活检显示均匀的短梭形细胞增殖，同时通过逆转录-聚合酶链反应检测到由染色体易位t(X;18)(p11;q11)导致的SYT-SSX融合基因。异环磷酰胺化疗联合姑息性放疗可有效抑制肺动脉和胸膜病变处肿瘤的生长，表明该方案可能对治疗无法切除的肺动脉滑膜肉瘤有效。
