Intravascular Synovial Sarcoma of the Pulmonary Artery with Massive Pleural Effusion: Report of a Case with a Favorable Response to Ifosfamide Chemotherapy and Palliative Radiation Therapy
Intravascular Synovial Sarcoma of the Pulmonary Artery with Massive Pleural Effusion: Report of a Case with a Favorable Response to Ifosfamide Chemotherapy and Palliative Radiation Therapy
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DOI：
10.2169/internalmedicine.54.3624
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发表时间：
2015-01-01
影响因子：
1.2
通讯作者：
Atagi, Shinji
中科院分区：
文献类型：
--
作者：
Maekura, Toshiya;Shimizu, Shigeki;Atagi, Shinji
滑膜肉瘤（SS）通常发生在关节旁软组织；然而，迄今为止报道的血管内SS病例很少。我们在此报告一例肺动脉SS伴大量胸腔积液的病例。胸膜病变活检显示均匀的短梭形细胞增殖，而在染色体易位t(X;18)（p11;q11）之前，使用逆转录-聚合酶链反应检测到SYT-SSX融合基因。异环磷酰胺化疗和姑息性放射治疗在减少肿瘤在肺动脉和胸膜病变中的生长方面是有效的，这表明该方案可能对治疗不可切除的肺动脉SS有用。
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.