Department of Pathology, Affiliated Anzhen Hospital of Capital Medical University, Beijing, China.
Department of Pathology, Affiliated Anzhen Hospital of Capital Medical University, Beijing, China.
Cardiovasc Pathol. 2021 Jan-Feb;50:107286. doi: 10.1016/j.carpath.2020.107286. Epub 2020 Sep 16.
Primary cardiac synovial sarcoma was an exceedingly rare tumor that less reported. The study investigated the clinicopathologic, immunohistochemical, and molecular features of primary cardiac synovial sarcoma.
A total of five cardiac synovial sarcoma cases were assessed and reviewed using H&E, immunohistochemical and fluorescence in situ hybridization staining methods. Clinicopathological data were retrospectively analyzed and followed up.
The cases occurred in four males and one female ranging in age from 23 to 48 years (mean, 32 years). The tumors were grossly large and solid (7.4-13.7 cm; mean 8.6 cm). Microscopically, clinical cases were biphasic (n = 2) and monophasic (n = 3) types and were diffusely immunoreactive for EMA, vimentin, and BCL-2. All cases demonstrated SS18 rearrangement by fluorescence in situ hybridization staining. Clinically, three patients died within 1 year after surgery, while one patient had bone metastasis and still carried the disease. One last patient underwent a heart transplant and survived without evidence of the disease.
Cardiac synovial sarcoma was an aggressive tumor whose differentiation may be a continuous and complex morphologic spectrum. SS18 rearrangement demonstration by fluorescence in situ hybridization was decisive in our study for differential diagnosis of cardiac synovial sarcoma and other tumors. Cardiac synovial sarcoma usually endured poor survival rates. Patients in advanced stages may undergo heart transplantation as a means of improving their survival rates.
原发性心脏滑膜肉瘤是一种罕见的肿瘤，报道较少。本研究旨在探讨原发性心脏滑膜肉瘤的临床病理、免疫组织化学和分子特征。
共评估了 5 例心脏滑膜肉瘤病例，并采用 H&E、免疫组织化学和荧光原位杂交染色方法进行了回顾性分析。分析了临床病理数据并进行了随访。
这些病例发生于 4 名男性和 1 名女性，年龄为 23-48 岁（平均 32 岁）。肿瘤大体上大而实性（7.4-13.7cm；平均 8.6cm）。镜下，临床病例为双相型（n=2）和单相型（n=3），均弥漫性表达 EMA、波形蛋白和 BCL-2。所有病例均通过荧光原位杂交染色显示 SS18 重排。临床上，3 例患者在手术后 1 年内死亡，1 例患者发生骨转移，仍患有该疾病。最后 1 例患者接受了心脏移植，无疾病证据，存活至今。
心脏滑膜肉瘤是一种侵袭性肿瘤，其分化可能是一个连续而复杂的形态学谱。在我们的研究中，荧光原位杂交显示 SS18 重排对于心脏滑膜肉瘤和其他肿瘤的鉴别诊断具有决定性意义。心脏滑膜肉瘤的总生存率通常较差。晚期患者可能需要接受心脏移植以提高生存率。
