He Wei-Wei, Huang Zhi-Xin, Wang Wen-Jing, Li Yu-Lei, Xia Qiu-Yuan, Qiu Yong-Bin, Shi Yi, Sun Hui-Ming
Department of Respiratory and Critical Care Medicine, Nanjing Yimin Hospital, Nanjing 210000, Jiangsu Province, China.
Department of Pathology, Jinling Hospital, Nanjing 210002, Jiangsu Province, China.
World J Clin Cases. 2022 May 26;10(15):5103-5110. doi: 10.12998/wjcc.v10.i15.5103.
Synovial sarcoma (SS) is an uncommon and highly malignant soft tissue sarcoma in the clinic, with primary pulmonary SS (PPSS) being extremely rare. Here, we describe the clinical characteristics, diagnosis, and treatment of a solitary PPSS case confirmed surgical resection and fluorescence in situ hybridization (FISH).
A 33-year-old man was admitted because of intermittent coughing and hemoptysis for one month, with lung shadows observed for two years. Whole-body positron emission tomography-computed tomography (PET-CT) revealed a solitary mass in the upper lobe of the right lung, with uneven radioactivity uptake and a maximum standardized uptake value of 5.6. The greyish-yellow specimen obtained following thoracoscopic resection was covered with small multi-nodulated structures and consisted of soft tissue. Hematoxylin and eosin staining revealed spindle-shaped malignant tumor cells. Immunohistochemistry indicated these tumor cells were CD99 and BCL-2-positive. Furthermore, the FISH test revealed synovial sarcoma translocation genetic reassortment, which confirmed the diagnosis of SS.
PPSS is extremely rare and tends to be misdiagnosed as many primary pulmonary diseases. PET-CT, histologic analysis, and FISH tests can be used to differentiate PPSS from other diseases. Surgical resection is regularly recommended for the treatment of solitary PPSS and is helpful for improving the prognosis.
滑膜肉瘤（SS）在临床上是一种罕见且高度恶性的软组织肉瘤，原发性肺滑膜肉瘤（PPSS）极为罕见。在此，我们描述一例经手术切除及荧光原位杂交（FISH）确诊的孤立性PPSS病例的临床特征、诊断及治疗情况。
一名33岁男性因间歇性咳嗽、咯血1个月入院，肺部阴影已发现2年。全身正电子发射断层扫描-计算机断层扫描（PET-CT）显示右肺上叶有一个孤立性肿块，放射性摄取不均匀，最大标准化摄取值为5.6。经胸腔镜切除获取的灰黄色标本表面有多个小结节状结构，由软组织构成。苏木精-伊红染色显示梭形恶性肿瘤细胞。免疫组化表明这些肿瘤细胞CD99和BCL-2呈阳性。此外，FISH检测显示滑膜肉瘤易位基因重排，确诊为SS。
PPSS极为罕见，容易被误诊为多种原发性肺部疾病。PET-CT、组织学分析及FISH检测可用于鉴别PPSS与其他疾病。对于孤立性PPSS，通常建议手术切除，这有助于改善预后。
