Background

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.

Methods

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.

Results

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.

Conclusion

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.

中文翻译：

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原发性心脏滑膜肉瘤：5例临床病理，免疫组织化学和分子遗传学研究。

背景

原发性心脏滑膜肉瘤是一种极为罕见的肿瘤，报道较少。该研究调查了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。

方法

使用H＆E，免疫组织化学和荧光原位杂交染色方法对总共5例心脏滑膜肉瘤病例进行了评估和审查。对临床病理资料进行回顾性分析和随访。

结果

该病例发生在四名男性和一名女性，年龄在23至48岁之间（平均32岁）。肿瘤大而坚实（7.4–13.7 cm；平均8.6 cm）。在显微镜下，临床病例为双相（n = 2）和单相（n = 3）类型，对EMA，波形蛋白和BCL-2具有弥漫性免疫反应。所有病例均通过荧光原位杂交染色证实SS18重排。临床上，有3例患者在手术后1年内死亡，而1例患者有骨转移并仍携带该病。最后一名患者接受了心脏移植手术，并在没有该疾病证据的情况下幸存。

结论

心脏滑膜肉瘤是一种侵袭性肿瘤，其分化可能是连续且复杂的形态学范围。通过荧光原位杂交证明SS18重排在我们的研究中对心脏滑膜肉瘤和其他肿瘤的鉴别诊断具有决定性意义。心脏滑膜肉瘤通常忍受较差的生存率。晚期患者可能会接受心脏移植，以提高其生存率。