title: 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.
abstract: 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.
authors.affiliation: Department of Internal Medicine, National Hospital Organization Kinki-chuo Chest Medical Center, Japan.
authors.forename: Toshiya
authors.initials: T
authors.lastname: Maekura
authors.forename: Shigeki
authors.initials: S
authors.lastname: Shimizu
authors.forename: Tomoya
authors.initials: T
authors.lastname: Kawaguchi
authors.forename: Masahiro
authors.initials: M
authors.lastname: Morimoto
authors.forename: Tomohisa
authors.initials: T
authors.lastname: Okuma
authors.forename: Hideo
authors.initials: H
authors.lastname: Matsui
authors.forename: Shinji
authors.initials: S
authors.lastname: Atagi
mesh_terms: D000328:Adult; D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage*; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage*; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D010996:Pleural Effusion / Q000188:drug therapy / Q000209:etiology*; D011651:Pulmonary Artery / Q000473:pathology*; D013584:Sarcoma, Synovial / Q000150:complications* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D014178:Translocation, Genetic; D019043:Vascular Neoplasms / Q000150:complications* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology
chemical_list: D018906:Antineoplastic Agents, Alkylating; D015514:Oncogene Proteins, Fusion; D007069:Ifosfamide