关键词: cardiac tumor pericardium solitary fibrous tumor

Mesh : Male Humans Aged, 80 and over Solitary Fibrous Tumors / diagnostic imaging surgery Mediastinum Tomography, X-Ray Computed Biopsy

来  源:   DOI:10.1002/cnr2.1698

Abstract:
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.
摘要:
孤立性纤维性肿瘤(SFT)是罕见的梭形细胞肿瘤,起源于内脏胸膜的间充质细胞。1931年,Klemperer等人首次将SFT描述为一个独特的实体。直到现在,关于胸膜外形式如心脏SFT的表现和行为,我们的数据有限。在这里,我们介绍了一例涉及心包的SFT,其中通过影像学检查进行诊断,然后进行活检。我们还回顾了涉及心脏和管理方法的SFT文献。
一名81岁男性出现进行性呼吸困难。胸部计算机断层扫描(CT)显示前纵隔有6.2×5.3cm的软组织肿块。CT血管造影的进一步成像显示与心包的茎状连接。肿块的活检显示梭形细胞对BCL-2,CD34和STAT6呈阳性,表明孤立性纤维瘤。对患者采取监测方法。
原发性心包肿瘤极为罕见,患病率为0.001%-0.007%。诊断SFT需要阳性CD34和BCL-2标志物。当前的建议是切除局部疾病,据记载,在良性疾病的情况下可以治愈,但对我们的患者进行了监视。
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