Biatrial primary synovial sarcoma of the heart.
Synovial sarcomas that primarily arise from the heart and pericardium are extremely rare, especially the ones involving both sides of the heart. To date, few cases have been reviewed in the literature. Our patient was a 36-year-old man who presented with primary biatrial synovial sarcoma that also involved the tricuspid annulus, heart valves, and interatrial septum. The tumor was debulked to relieve the atrioventricular obstruction. Molecular analysis confirmed the diagnosis of synovial sarcoma with positive t (X;18) SYT-SSX gene fusion. The patient is currently receiving chemotherapy. From our review of the 20 cases thus far reported in the English-language medical literature, the tumor more frequently affects young male patients, and carries a poor prognosis. Early detection is difficult because of the aggressive nature of the tumor. Like other sarcomas, wide surgical resection remains the mainstay of therapy. Adjuvant radiation therapy for local recurrence and chemotherapy for control of systemic disease may have some beneficial effect on overall survival, but the benefit is likely limited.
Zhao
Qiong
Q
Section of Cardiology, Department of Medicine, The University of Illinois at Chicago School of Medicine, Chicago, Illinois 60612, USA. qiongzhao@yahoo.com
Geha
Alexander S
AS
Devries
Stephen R
SR
Tshibaka
Cimenga
C
Wu
Shou-jin
SJ
Lavi
Nimrod
N
Massad
Malek
M
Chomka
Eva V
EV
D000328:Adult; D006338:Heart Neoplasms / Q000000981:diagnostic imaging* / Q000473:pathology*; D006801:Humans; D008297:Male; D013584:Sarcoma, Synovial / Q000000981:diagnostic imaging* / Q000473:pathology*; D014463:Ultrasonography