小肠单相滑膜肉瘤的治疗
Management of monophasic synovial sarcoma of the small intestine.
作者信息
Eriksen Calvin, Burns Lance, Bohlke Angela, Haque Salima, Slakey Douglas P
机构信息
Departments of Surgery and Pathology, Tulane University School of Medicine, New Orleans, Louisiana, USA.
出版信息
JSLS. 2010 Jul-Sep;14(3):421-5. doi: 10.4293/108680810X12924466006846.
BACKGROUND
Reports of primary intraabdominal synovial sarcomas are extremely rare.
METHODS
A literature review using PubMed was performed. A retrospective review of the one known case at our institution was completed.
RESULTS
Even the most experienced pathologists report that synovial sarcomas can be very difficult to diagnose correctly. One cytogenic abnormality that is common (> 90%) and pathognomonic for synovial sarcoma is a characteristic chromosomal translocation resulting in the SYT/SSX fusion gene. Wide regional excision has been performed for intraabdominal sarcoma, with improved results. Our patient is more than 24 months with no evidence of recurrent or metastatic disease.
CONCLUSIONS
The prognosis for patients with intraabdominal synovial sarcoma remains poor. However, wide regional excision may allow for prolonged disease-free survival.
背景
原发性腹腔内滑膜肉瘤的报道极为罕见。
方法
利用PubMed进行文献综述。对我们机构已知的1例病例进行回顾性分析。
结果
即使是最有经验的病理学家也报告说，滑膜肉瘤很难正确诊断。一种常见（>90%）且对滑膜肉瘤具有诊断意义的细胞遗传学异常是特征性的染色体易位，导致SYT/SSX融合基因。对于腹腔内肉瘤已进行广泛区域切除，效果有所改善。我们的患者已存活超过24个月，无复发或转移性疾病迹象。
结论
腹腔内滑膜肉瘤患者的预后仍然很差。然而，广泛区域切除可能会延长无病生存期。
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