We report a 49-year-old Southeast Asian woman diagnosed with metastatic primary right atrial angiosarcoma (PCA) and the difficulties encountered in this diagnosis and its subsequent management. Diagnosis of PCA is often delayed due to non-specific clinical presentation of patients. These tumours often present once metastatic spread has occurred, restricting treatment options and leading to very poor prognosis. Patients undergo a multidisciplinary team (MDT) approach involving chemotherapy, radiotherapy and, if eligible, surgery, but evidence-based treatment guidelines have yet to be established due to the rarity of the tumour.
BurkeA, VirmaniR, RosaiJ. Atlas of Tumor Pathology: Tumors of the Heart and Great Vessels 3rd series. Washington DC: American Registry of Pathology; 1995.
2.
LahatG, DhukaAR, LahatS. Outcome of locally recurrent and metastatic angiosarcoma. Ann Surg Oncol2009; 16: 2502–9.
VirmaniR.. Tumors and tumor-like conditions of the heart. In: SilverMD, GotliebAI, SchoenFJ.Cardiovascular Pathology 3rd ed. Churchill Livingstone; 2001;583–605.
7.
WangH, ShiJ, LiuH. Clinical and diagnostic features of angiosarcoma with pulmonary metastases: a retrospective observational study. Medicine (Baltimore)2017; 96: e8033.
8.
YangHS, SenguptaS, UmlandMM. Primary cardiac angiosarcoma evaluated with contrast two-dimensional and real-time three-dimensional echocardiography. Eur J Echocardiogr2008; 9: 733–8.
9.
GaballahAH, JensenCT, PalmquistS. Angiosarcoma: clinical and imaging features from head to toe. Br J Radiol2017; 90:20170039.
10.
NayarS, NayarPG, CherianKM. Angiosarcoma presenting as syncope. Asian Cardiovasc Thorac Ann2008; 16: 154–6.
11.
RandallMB, GeisingerKR. Angiosarcoma of the heart: pericardial fluid cytology. Diagn Cytopathol1990; 6: 58–62.
12.
BoumaW, LexisCP. Successful surgical excision of primary right atrial angiosarcoma. J Cardiothorac Surg2011; 6: 1–6.
13.
CioffiA, ReichertS, AntonescuCR. Angiosarcomas and other sarcomas of endothelial origin. Hematol Oncol Clin North Am2013; 27: 975–88.
14.
PervaizN, ColterjohnN, FarrokhyarF. A systematic meta-analysis of randomized controlled trials of adjuvant chemotherapy for localized resectable soft-tissue sarcoma. Cancer2008; 113: 573–81.
