- Technologist presentation
- Open Access
An unusual cause of right heart dilatation
© Han et al. 2016
- Published: 27 January 2016
- Pulmonary Embolism
- Pulmonary Valve
- Right Ventricular Outflow Tract
- Myofibroblastic Differentiation
- Pulmonary Valve Replacement
There is a large mass causing near occlusion of the main PA. This mass extends distally into proximal left and right PA. This mass also extends proximally into pulmonary valve and proximal posterior aspect of the RV out flow tract.
The patient underwent complete endarterectomy, pulmonary valve replacement and PA reconstruction using a bifurcated Gelweave graft. She recovered well and was discharged home subsequently. Histology showed intimal sarcoma with focal myofibroblastic differentiation.
Pulmonary artery sarcomas are exceedingly rare. Other important and more common differential diagnoses to be considered include pulmonary embolism (PE) or tumor embolism from pulmonary or other malignancies. Pulmonary artery sarcoma mimicking as PE, pulmonary artery aneurysm or Takayasu's disease has been reported in the literature. In our case, the absence of thromboembolism risk factors, together with heterogeneous tumor enhancement and invasion into the wall of main pulmonary artery as well as its branches are suggestive of a malignant process instead of PE.
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