title: A poorly differentiated synovial sarcoma arising from the pulmonary valve.
abstract: Synovial sarcoma originating in the pulmonary valve is extremely rare. Herein, we report a poorly differentiated synovial sarcoma arising from this peculiar location in a 17-year-old Chinese boy. Histologically, this tumor was entirely poorly differentiated with uniform small round cell morphology, and it exhibited prominent myxoid change in some areas. The diagnosis was confirmed by the presence of SS18 rearrangement and identification of the SS18-SSX1 fusion transcript. To the best of our knowledge, the present case is the first published example of synovial sarcoma occurring in the pulmonary valve. Additionally, this is the first case showing entirely uniform small round cell morphology without classic areas of synovial sarcoma.
authors.affiliation: Department of Pathology, West China Hospital, Sichuan University, Chengdu 610041, Sichuan, China.
authors.forename: Lijuan
authors.initials: L
authors.lastname: Yin
authors.forename: Min
authors.initials: M
authors.lastname: Chen
authors.forename: Feng
authors.initials: F
authors.lastname: Ye
authors.forename: Hong
authors.initials: H
authors.lastname: Bu
authors.forename: Hongli
authors.initials: H
authors.lastname: Bai
authors.forename: Jianqun
authors.initials: J
authors.lastname: Yu
authors.forename: Hongying
authors.initials: H
authors.lastname: Zhang
mesh_terms: D000293:Adolescent; D001706:Biopsy; D002454:Cell Differentiation; D048430:Cell Shape; D015321:Gene Rearrangement; D006338:Heart Neoplasms / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008279:Magnetic Resonance Imaging; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D011518:Proto-Oncogene Proteins / Q000235:genetics; D011664:Pulmonary Valve / Q000473:pathology* / Q000601:surgery; D012097:Repressor Proteins / Q000235:genetics; D013584:Sarcoma, Synovial / Q000235:genetics / Q000473:pathology* / Q000601:surgery; D016896:Treatment Outcome
chemical_list: D015514:Oncogene Proteins, Fusion; D011518:Proto-Oncogene Proteins; D012097:Repressor Proteins; C093961:SS18 protein, human; C587877:SS18-SSX1 fusion protein
keywords: Pulmonary valve; Synovial sarcoma