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Primary angiosarcoma of superior vena cava: an unexpected diagnosis after an oncological emergency
  1. Fábio Rêgo Salgueiro1,
  2. Inês Vieira2,
  3. Inês Gomes1 and
  4. Maja Petrova3
  1. 1Medical Oncology, Instituto Português de Oncologia de Coimbra Francisco Gentil EPE, Coimbra, Portugal
  2. 2Endocrinology and Metabolism, Centro Hospitalar Universitário de Coimbra EPE, Coimbra, Portugal
  3. 3Internal Medicine, Centro Hospitalar Universitário de Coimbra EPE, Coimbra, Portugal
  1. Correspondence to Dr Fábio Rêgo Salgueiro; f.r.salgueiro{at}


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.

  • oncology
  • pathology

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  • Contributors FRS and IV contributed equally to this work. FRS, IV and MP were involved in the patient’s care. FRS and IV had the initiative to write this article, explained the implications of it to the patient and obtained his consent. They were also responsible for patient information collection, analysis and interpretation, as well as the literature review and discussion of global health problems. IG collaborated in organising the information, in the literature review and discussion, in writing important parts of the manuscript and revising it extensively. FRS, IV and IG also wrote and edited the manuscript and critically reviewed the scientific and formal content. All the authors approved the final version of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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