Medical Oncology, Instituto Português de Oncologia de Coimbra Francisco Gentil EPE, Coimbra, Portugal
Endocrinology and Metabolism, Centro Hospitalar Universitário de Coimbra EPE, Coimbra, Portugal.
BMJ Case Rep. 2021 Apr 16;14(4):e241266. doi: 10.1136/bcr-2020-241266.
Angiosarcoma (AS) is a rare malignant tumour representing 1%-2% of all sarcomas. Primary AS of superior vena cava (SVC) was reported in two cases worldwide. We report a 69-year-old woman with neck discomfort, headache and dyspnoea for 3 months. CT angiography showed thrombosis in SVC and brachiocephalic veins resulting in an SVC syndrome. The patient began anticoagulant therapy and underwent balloon angioplasty with clinical improvement. Additionally, a positron emission tomography scan confirmed the presence of a mediastinal mass involving the SVC locally. The tumour was excised and a prosthesis was placed on the SVC. Histology revealed a heterogeneous tumour matrix, either myxoid and composed by fusiform cells with vimentin, homogeneous CD31 and a 30% Ki67 immunoexpression, supporting the diagnosis of an AS. Due to multiple complications, the patient never started chemotherapy, and after tumour recurrence, she died within 5 months after diagnosis.
血管肉瘤（AS）是一种罕见的恶性肿瘤，占所有肉瘤的 1%-2%。全世界仅报告过两例原发性上腔静脉（SVC）AS。我们报告了一例 69 岁女性，颈部不适、头痛和呼吸困难 3 个月。CT 血管造影显示 SVC 和头臂静脉血栓形成导致 SVC 综合征。患者开始抗凝治疗，并进行了球囊血管成形术，临床症状得到改善。此外，正电子发射断层扫描（PET）扫描证实纵隔内存在累及 SVC 的肿块。肿瘤被切除，并在 SVC 上放置了一个假体。组织学显示肿瘤基质不均匀，黏液样，由梭形细胞组成，波形蛋白阳性，CD31 均匀表达，Ki67 免疫组化表达为 30%，支持 AS 的诊断。由于多种并发症，患者从未开始化疗，在肿瘤复发后，她在诊断后 5 个月内死亡。
