Oliveira Cristiano Claudino, de Moraes Marcelo Padovani Toledo, Colby Thomas, Oliveira Gilmar Felisberto, Hasimoto Erica Nishida, Cataneo Daniele Cristina, Cataneo Antônio José Maria, De Faveri Julio
Department of Pathology - Botucatu School of Medicine - Universidade Estadual Paulista "Julio de Mesquista Filho", Botucatu/SP - Brazil .
Department of Laboratory Medicine and Pathology - Mayo Clinic., Phoenix/AZ, USA .
Autops Case Rep. 2016 Dec 30;6(4):35-40. doi: 10.4322/acr.2016.053. eCollection 2016 Oct-Dec.
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.
孤立性纤维瘤(SFT)是一种间叶性肿瘤,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岁女性,她出现阻塞性呼吸道症状4年。胸部计算机断层扫描和支气管镜检查显示,在气管和左主支气管之间有一个阻塞性息肉样病变,伴有左肺远端肺不张。对该病变进行了切除,肉眼可见肿块呈椭圆形,有包膜,质地坚硬,大小为2.3×1.7×1.5厘米。组织学显示为低级别间叶性梭形细胞瘤,细胞密度交替,有黏液样区域和成熟脂肪组织灶,以及壁不规则的血管。免疫组织化学研究显示CD34、CD99和BCL2呈阳性。诊断为位于不寻常部位的SFT。肿瘤切除后患者症状缓解,未发现明显的全身问题。SFT主要影响成年人,通常病程良性;然而,其行为难以预测。坏死和有丝分裂活动的存在可能预示预后不良。支气管内SFT很少见,但应与其他部位的SFT一样进行评估和监测,并进行长期随访。