Ngahane B H Mbatchou, Baudrand H, Traverse-Glehen A, Freymond N, Guibert B, Pacheco Y, Devouassoux G
Service de pneumologie, bâtiment 5F, centre hospitalier Lyon-Sud, Hospices Civils de Lyon, 165, chemin du Grand-Revoyet, 69495 Pierre-Bénite cedex, France; Université Claude-Bernard, 69008 Lyon, France.
Rev Mal Respir. 2010;27(1):93-7. doi: 10.1016/j.rmr.2009.11.006. Epub 2009 Nov 28.
Synovial sarcoma is an uncommon tumour and thoracic involvement is rare and of varying location. Clinical characteristics are dominated by pain, with a slow progression over years. Pathological and immuno-histochemical characteristics are helpful in the diagnosis but a specific translocation between chromosomes X and 18 is crucial for confirmation. Extensive surgical resection is required for cure, combined with adjuvant radiotherapy in the presence of adverse prognostic factors.
We report a case of synovial sarcoma of the chest wall, responsible for chronic local pain for several years, presenting as an acute pleuropneumonitis in a 21-year-old patient. In view of the large size of the tumour, associated with a high proliferation index (Ki-67), a surgical resection was performed, together with local adjuvant radiotherapy.
This case report reviews synovial sarcoma and underlines the difficulties and requirements of both diagnostic strategy and therapeutic management. Among them, an initial systematic review of prognostic factors (tumour size, mitotic activity, proliferation index, SYT-SSX type fusion, histological grade) is crucial to determine the therapeutic options.
滑膜肉瘤是一种罕见肿瘤，胸部受累情况罕见且部位各异。临床特征以疼痛为主，多年来进展缓慢。病理及免疫组化特征有助于诊断，但X染色体与18号染色体之间的特定易位对于确诊至关重要。治愈需要广泛手术切除，存在不良预后因素时联合辅助放疗。
我们报告一例胸壁滑膜肉瘤病例，该病例导致一名21岁患者出现数年慢性局部疼痛，并表现为急性胸膜肺炎。鉴于肿瘤体积较大，且增殖指数（Ki-67）较高，遂进行了手术切除，并联合局部辅助放疗。
本病例报告回顾了滑膜肉瘤，并强调了诊断策略及治疗管理中的困难与要求。其中，对预后因素（肿瘤大小、有丝分裂活性、增殖指数、SYT-SSX型融合、组织学分级）进行初步系统评估对于确定治疗方案至关重要。
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