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胸部原发性滑膜肉瘤:影像学与临床病理对照

Primary synovial sarcoma of the chest: radiographic and clinicopathologic correlation.

作者信息

Duran-Mendicuti Alejandra, Costello Philip, Vargas Sara O

机构信息

Department of Radiology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA 02115, USA.

出版信息

J Thorac Imaging. 2003 Apr;18(2):87-93. doi: 10.1097/00005382-200304000-00006.

DOI:10.1097/00005382-200304000-00006
PMID:12700482
Abstract

Primary synovial sarcoma of the thorax is rare. Origin of thoracic synovial sarcoma in the pleura or lung was first described only 7 years ago. Radiologic characteristics of this disease have not yet been studied in a formal series. The authors sought to define the radiologic features of primary thoracic synovial sarcoma and to correlate the findings with clinical and pathologic features. They examined clinical, radiologic, and pathologic features of five patients with primary synovial sarcoma of the chest. Radiologic evaluation included conventional radiographs, computed tomographic scans, and magnetic resonance images of the chest. Patients included three men and two women who ranged in age from 28 to 40 years. Primary tumors involved the chest wall (n = 2), lung (n = 1), or both (n = 2). Chest pain was the most common presenting symptom. Although conventional radiographs often showed the lesions to be ill defined, computed tomographic scans showed well-defined masses in every case. Heterogeneous enhancement and an absence of calcification were also seen. Pathologic evaluation demonstrated synovial sarcoma with equal distribution between the monophasic and biphasic variants. The chromosomal translocation X;18 was demonstrated in four of four cases tested. All patients were treated by resection. Recurrence was demonstrated radiologically in four patients at 2 to 14 months. All patients were alive at 9 to 58 months of follow-up. The authors conclude that primary synovial sarcoma of the chest occurs in young adults, most commonly presenting with chest pain. It is characterized radiologically by a heterogeneously enhancing well-defined mass without calcifications.

摘要

原发性胸部滑膜肉瘤较为罕见。胸膜或肺部的胸部滑膜肉瘤起源直到7年前才首次被描述。尚未对该疾病的放射学特征进行正式系列研究。作者试图明确原发性胸部滑膜肉瘤的放射学特征,并将这些发现与临床和病理特征相关联。他们检查了5例原发性胸部滑膜肉瘤患者的临床、放射学和病理特征。放射学评估包括胸部的传统X线片、计算机断层扫描(CT)和磁共振成像(MRI)。患者包括3名男性和2名女性,年龄在28至40岁之间。原发性肿瘤累及胸壁(2例)、肺(1例)或两者(2例)。胸痛是最常见的首发症状。尽管传统X线片常显示病变边界不清,但CT扫描在每例中均显示边界清晰的肿块。还可见不均匀强化且无钙化。病理评估显示滑膜肉瘤在单相型和双相型变体中分布相等。在检测的4例病例中,有4例证实存在染色体易位X;18。所有患者均接受了手术切除。4例患者在2至14个月时经放射学检查证实复发。在9至58个月的随访中,所有患者均存活。作者得出结论,原发性胸部滑膜肉瘤发生于年轻人,最常见的表现为胸痛。其放射学特征为边界清晰、不均匀强化且无钙化的肿块。

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