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CP-EBUS-TBNA在马富西综合征患者软骨肉瘤诊断中的应用。

The use of CP-EBUS-TBNA in the diagnosis of chondrosarcoma in a patient With Maffucci syndrome.

作者信息

Wang Ruobing, Folch Erik, Paul Manju, Maskey Ashish, Allard Felicia, Majid Adnan

机构信息

*Department of Surgery, Beth Israel Deaconess Medical Center, Division of Thoracic Surgery and Interventional Pulmonology, Harvard Medical School †Department of Pediatrics, Division of Respiratory Diseases, Boston Children's Hospital, Harvard Medical School ‡Department of Pathology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA.

出版信息

J Bronchology Interv Pulmonol. 2014 Apr;21(2):177-80. doi: 10.1097/LBR.0000000000000069.

Abstract

We report a case of a 52-year-old white woman with Maffucci syndrome, a rare skeletal disorder characterized by multiple cartilage-forming tumors (enchondromas). She was referred for evaluation of an enlarging pulmonary nodule. Her positron emission tomography-computed tomography scan revealed a low-density, tubular lesion in the superior segment of the left lower lobe of the lung. She underwent a convex-probe endobronchial ultrasound (CP-EBUS)-guided transbronchial needle aspiration (TBNA) that confirmed metastatic chondrosarcoma. Although CP-EBUS-TBNA has become an established tool for diagnosing and staging of non-small cell lung cancer, analysis of samples from rare tumors such as sarcomas by cytology alone is often inadequate and histologic confirmation using more invasive procedures is often required. Interestingly in our case, the diagnosis of chondrosarcoma was established from the cytology specimen obtained with a 21-G needle without the need for further tissue sampling. Moreover, the location of the lesion was the superior segment of the left lower lobe, an area typically not accessible by CP-EBUS, due to the size of the airway. Considering together, the successful diagnosis of a rare tumor such as sarcomas by CP-EBUS-TBNA modality indicates the growing importance of this minimally invasive technology.

摘要

我们报告一例52岁的白人女性患有马富西综合征,这是一种罕见的骨骼疾病,其特征为多发性软骨形成肿瘤(内生软骨瘤)。她因肺部结节增大前来评估。她的正电子发射断层扫描 - 计算机断层扫描显示左肺下叶上段有一个低密度管状病变。她接受了凸探头支气管内超声(CP - EBUS)引导下的经支气管针吸活检(TBNA),确诊为转移性软骨肉瘤。尽管CP - EBUS - TBNA已成为诊断和分期非小细胞肺癌的既定工具,但仅通过细胞学分析来自肉瘤等罕见肿瘤的样本往往并不充分,通常需要采用更具侵入性的程序进行组织学确认。有趣的是,在我们的病例中,通过21G针获取的细胞学标本确诊为软骨肉瘤,无需进一步组织采样。此外,病变位于左肺下叶上段,由于气道大小,该区域通常无法通过CP - EBUS到达。综合考虑,通过CP - EBUS - TBNA方式成功诊断肉瘤等罕见肿瘤表明了这种微创技术日益重要。

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