Panigrahi Manoj Kumar, Pradhan Gourahari, Sahoo Nibedita, Mishra Pritinanda, Patra Susama, Mohapatra Prasanta Raghab
Department of Pulmonary Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
Department of Pathology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India.
J Cancer Res Ther. 2018 Apr-Jun;14(3):481-489. doi: 10.4103/0973-1482.204883.
Synovial sarcoma (SS) is a malignant mesenchymal tumor with variable epithelial differentiation that affects mostly young adults and can arise at any anatomic site. Primary intrathoracic SS is very rare accounting for <0.5% of all lung tumors. Most commonly, it arises from the lung followed by pleura and mediastinum. Primary pulmonary SS (PPSS) affects both sexes equally with no preference for any hemithorax. The morphology, immunostaining properties, cytogenetic features, and management strategy of PPSS are similar to that of soft tissue SS. Histologically, there are two main types of SS - monophasic and biphasic with a feature of poor differentiation seen in both types. Most patients present with large intrathoracic masses with or without ipsilateral pleural effusion. Bone invasion or mediastinal adenopathy is very rare. SS is characterized by a specific chromosomal translocation producing SS18-SSX fusion gene in more than 90% of cases. Identification of this fusion gene remains the gold standard for the diagnosis in the presence of consistent histology and immunophenotype. Multimodality treatment including wide excision, chemotherapy, and radiotherapy is the mainstay of therapy. SS is relatively chemosensitive, and ifosfamide-based regimen showed improved survival in metastatic disease. Generally, SS is considered as high-grade tumors with a poor prognosis. Novel therapies targeted at fusion oncogene, SS18-SSX-derived peptide vaccine, epidermal growth factor receptor, and vascular endothelial growth factor are the future hope in SS. We describe a prototype case and present an elaborate review on primary SS of lung.
滑膜肉瘤（SS）是一种具有可变上皮分化的恶性间充质肿瘤，主要影响年轻人，可发生于任何解剖部位。原发性胸内滑膜肉瘤非常罕见，占所有肺部肿瘤的比例不到0.5%。最常见的是起源于肺，其次是胸膜和纵隔。原发性肺滑膜肉瘤（PPSS）在两性中发病率相同，对任何一侧胸腔均无偏好。PPSS的形态学、免疫染色特性、细胞遗传学特征和治疗策略与软组织滑膜肉瘤相似。组织学上，滑膜肉瘤主要有两种类型——单相型和双相型，两种类型均具有分化不良的特征。大多数患者表现为胸腔内大肿块，伴有或不伴有同侧胸腔积液。骨侵犯或纵隔淋巴结肿大非常罕见。滑膜肉瘤的特征是一种特定的染色体易位，在超过90%的病例中产生SS18-SSX融合基因。在组织学和免疫表型一致的情况下，识别这种融合基因仍然是诊断的金标准。多模式治疗包括广泛切除、化疗和放疗是主要的治疗方法。滑膜肉瘤对化疗相对敏感，基于异环磷酰胺的方案在转移性疾病中显示出存活率提高。一般来说，滑膜肉瘤被认为是预后较差的高级别肿瘤。针对融合癌基因、SS18-SSX衍生肽疫苗、表皮生长因子受体和血管内皮生长因子的新型疗法是滑膜肉瘤治疗的未来希望。我们描述了一个典型病例，并对原发性肺滑膜肉瘤进行了详细综述。
