Mlika M, Berraies A, Boudaya M S, Hamzaoui A, El Mezni F
Pathologica. 2014 Dec;106(4):335-7.
Vascular tumours of the mediastinum are rare, accounting for 1-2% of all mediastinal tumours in this location. Angiosarcomas are most often encountered as sporadic lesions, typically in the scalp or face of elderly patients. However, they can occur in any anatomic site. Mediastinal angiosarcomas (MA) are very rare with less than 50 cases reported.
The authors describe the case of a 38-year-old woman whose past medical history was consistent for a MA that was diagnosed in 2003. This tumour was treated by complete surgical resection followed by radiation therapy and chemotherapy. Diagnosis was based on histologic examination. In 2011, the patient presented a pleural localisation of the angiosarcoma and died one month after admission, 8 years after diagnosis of the MA.
MA is a very rare tumour causing a diagnostic dilemma. Clinical and radiologic findings are non-specific, and final diagnosis is based on histologic examination. The case described is unusual considering the long period of survival, which may be explained by the treatment modalities associating complete surgical resection, chemotherapy and radiation therapy.
纵隔血管肿瘤较为罕见，占该部位所有纵隔肿瘤的1% - 2%。血管肉瘤最常表现为散发性病变，典型地发生于老年患者的头皮或面部。然而，它们可发生于任何解剖部位。纵隔血管肉瘤（MA）极为罕见，报道的病例少于50例。
作者描述了一名38岁女性的病例，其既往病史符合2003年诊断的MA。该肿瘤通过完整手术切除，随后进行放疗和化疗进行治疗。诊断基于组织学检查。2011年，患者出现血管肉瘤的胸膜定位，并在诊断MA 8年后入院1个月后死亡。
MA是一种极为罕见的肿瘤，会导致诊断困境。临床和放射学表现不具有特异性，最终诊断基于组织学检查。考虑到较长的生存期，所描述的病例并不常见，这可能是由于联合了完整手术切除、化疗和放疗的治疗方式所致。
