Pulmonary Artery Angiosarcoma

Physiology

  • Pulmonary HTN due to obstruction of PA by tumor

In tumor obstruction, a tumor grows into the central pulmonary arteries, with additional appositional thrombosis leading to a progressive obstruction of proximal pulmonary arteries and PH. Such cases are due principally to pulmonary artery sarcomas, occur rarely, and are usually rapidly fatal (132,133). The differential diagnosis with CTEPH can be difficult. Computed tomography and magnetic resonance imaging angiography are the most useful diagnostic modalities for differentiation between tumor and thrombotic material (132,134,135).


Clinical Presentation


References

  • 132 Mayer E, Kriegsmann J, Gaumann A, et al. Surgical treatment of pulmonary artery sarcoma. J Thorac Cardiovasc Surg 2001;121: 77– 82.
  • 133 Anderson MB, Kriett JM, Kapelanski DP, Tarazi R, Jamieson SW. Primary pulmonary artery sarcoma: a report of six cases. Ann Thorac Surg 1995;59:1487–90.
  • 134 Kim HK, Choi YS, Kim K, et al. Surgical treatment for pulmonary artery sarcoma. Eur J Cardiothorac Surg 2008;33:712–6.
  • 135 Ishiguro T, Kasahara K, Matsumoto I, et al. Primary pulmonary artery sarcoma detected with a pulmonary infarction. Intern Med 2007;46:601–4