Department of Cardiac Surgery, CIMS Hospital, Ahmedabad, Gujarat, India.
J Card Surg. 2021 Aug;36(8):2992-2995. doi: 10.1111/jocs.15631. Epub 2021 May 12.
Primary cardiac synovial sarcoma is an exceedingly rare soft tissue malignant tumor, involving either the pericardium or chambers, with a striking male predominance, prevalently seen in the fourth decade. These tumours most commonly present as breathlessness on exertion. These patients readily exhibit gastrointestinal and systemic symptoms but sometimes the presentation can be fallacious.
A 37-year old male presented with fever, breathlessness on exertion and hematuria who was managed for dengue feverat another hospital. Patient also had features of right heart failure and congestive hepatomegaly. Patient was evaluated thoroughlyand diagnosed as right atrial tumour mass for which surgical resection was done.
The tumour mass was diagnosed as right atrial biphasic synovial sarcoma after immunohistochemistry. Patient also had thrombocytopenia which was due to increased consumption of platelets.
Right heart biphasic synovial sarcoma, arising from the tricuspid valve in association with thrombocytopenia and right heart failure is an extremely rare entity and that can have a deceptive presentation.
原发性心脏滑膜肉瘤是一种极为罕见的软组织恶性肿瘤，涉及心包或心腔，男性发病显著多于女性，主要见于 40 岁左右。这些肿瘤最常见的表现是活动时呼吸困难。这些患者常伴有胃肠道和全身症状，但有时表现可能会有误导。
一名 37 岁男性因发热、活动时呼吸困难和血尿在另一家医院就诊，被诊断为登革热。患者还存在右心衰竭和充血性肝肿大的表现。患者经过全面评估，被诊断为右心房肿瘤，随后进行了手术切除。
肿瘤经免疫组化检查后被诊断为右心房双相滑膜肉瘤。患者还伴有血小板减少症，这是由于血小板消耗增加所致。
源于三尖瓣的右心双相滑膜肉瘤，伴发血小板减少症和右心衰竭，是一种极其罕见的实体肿瘤，其表现可能具有欺骗性。
