School of Medicine, Texas Tech University Health Science Center, Lubbock, Texas, USA.
Department of Internal Medicine, Texas Tech University Health Science Center, Lubbock, Texas, USA.
Cancer Rep (Hoboken). 2022 Nov;5(11):e1698. doi: 10.1002/cnr2.1698. Epub 2022 Aug 30.
Solitary fibrous tumor (SFT) are rare spindle cell tumors originating from the mesenchymal cells mostly from the visceral pleura. SFT was first described as a distinct entity in 1931 by Klemperer et al. Until now, we have limited data regarding the manifestation and behavior of extra pleural forms such as cardiac SFT. Here we present a case of SFT involving the pericardium where the diagnosis was made by imaging followed by biopsy findings. We also review the literature of SFT involving the heart and the management approaches.
An 81-year-old male presented with progressive dyspnea. Computed tomography (CT) of the chest showed a 6.2 × 5.3 cm soft tissue mass in the anterior mediastinum. Further imaging with CT angiogram showed a stalk-like connection to the pericardium. A biopsy of the mass showed spindle cells positive for BCL-2, CD34, and STAT 6, indicative of a solitary fibrous tumor. A surveillance approach was adopted for the patient.
Primary pericardial tumors are exceedingly rare, with a prevalence rate of 0.001%-0.007%. Diagnosing a SFT requires a positive CD34 and BCL-2 marker. The current recommendation is resection of localized disease which has been documented to be curative in cases of benign disease however our patient was put on surveillance.
孤立性纤维瘤（SFT）是一种罕见的梭形细胞肿瘤，起源于间充质细胞，主要来自内脏胸膜。SFT 于 1931 年由 Klemperer 等人首次描述为一种独特的实体。到目前为止，我们对于胸膜外形式（如心脏 SFT）的表现和行为的了解有限。在这里，我们报告了一例累及心包的 SFT 病例，通过影像学检查和活检结果做出了诊断。我们还回顾了涉及心脏的 SFT 文献和管理方法。
一名 81 岁男性因进行性呼吸困难就诊。胸部计算机断层扫描（CT）显示前纵隔有一个 6.2×5.3cm 的软组织肿块。进一步的 CT 血管造影显示与心包有一个茎状连接。肿块的活检显示梭形细胞 BCL-2、CD34 和 STAT6 阳性，提示为孤立性纤维瘤。对患者采取了监测方法。
原发性心包肿瘤极为罕见，患病率为 0.001%-0.007%。诊断 SFT 需要 CD34 和 BCL-2 标志物阳性。目前的建议是切除局限性疾病，已证明对良性疾病是治愈性的，但我们的患者被置于监测中。
