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Endobronchial solitary fibrous tumor

机译:支气管内孤立性纤维瘤

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Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that appears primarily in the pleura and rarely in intrapulmonary or endobronchial topography. The authors report the case of a 47-year-old woman who presented obstructive respiratory symptoms for 4 years. The chest computed tomography and bronchoscopy showed an obstructive polypoid lesion located between the trachea and the left main bronchus associated with distal atelectasis of the left lung. A resection of the lesion was performed and, macroscopically, the mass was oval, encapsulated, and firm, measuring 2.3 × 1.7 × 1.5 cm. Histology revealed low-grade mesenchymal spindle cell neoplasm, with alternating cellularity, myxoid areas, and mature adipose tissue outbreaks, as well as blood vessels with irregular walls. The immunohistochemical study was positive for CD34, CD99, and BCL2. The diagnosis was SFT in an unusual topography. The patient’s symptoms remitted after tumor excision, and no systemic problems were evident. SFTs primarily affect adults and often follow a benign course; however, their behavior is unpredictable. The presence of necrosis and mitotic activity may portend a poor prognosis. Endobronchial SFTs are rare but should be evaluated and monitored similar to SFTs at other sites, with a long-term follow-up.
机译:孤立性纤维性肿瘤(SFT)是一种间质性肿瘤,主要出现在胸膜,很少出现在肺内或支气管内。作者报告了一例47岁的妇女,该妇女出现阻塞性呼吸道症状长达4年。胸部计算机断层扫描和支气管镜检查显示,位于气管和左主支气管之间的阻塞性息肉样病变与左肺远端肺不张相关。切除病灶,肉眼可见肿块为椭圆形,被包封且牢固,大小为2.3×1.7×1.5 cm。组织学显示低度间充质梭形细胞瘤,具有交替的细胞性,粘液样区域和成熟的脂肪组织暴发,以及具有不规则壁的血管。免疫组化研究对CD34,CD99和BCL2呈阳性。诊断是在异常地形中的SFT。肿瘤切除后患者的症状缓解了,没有明显的系统性问题。 SFT主要影响成年人,并且通常遵循良性发展过程;但是,它们的行为是不可预测的。坏死和有丝分裂活动可能预示不良预后。支气管内SFT很少见,但应与其他部位的SFT相似地进行评估和监测,并进行长期随访。

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