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Cardiovascular Images

MRI of Intimal Sarcoma of the Pulmonary Arteries

Pia K. Schuler, MD; Achim Weber, MD; Peter K. Bode, MD; Michael Neuhaus, MD; René Prêtre, MD; Rolf Jenni, MD, MSEE and Jürg Schwitter, MD, FESC

From the Clinic for Cardiology (P.K.S., R.J., J.S.), the Institute of Surgical Pathology (A.W., P.K.B.), and the Clinic for Cardiovascular Surgery (R.P.),University Hospital Zurich, Switzerland; and the Clinic for Internal Medicine (M.N.), Kantonsspital Baden, Switzerland.

Correspondence to Pia Schuler, MD, University Hospital Zurich, Cardiology Clinics, Raemistrasse 100, CH-8091 Zurich, Switzerland. E-mail schulerp@gmx.de

An extract of the first 250 words of the full text is provided, because this article has no abstract.

A 38-year-old woman presented with left lower chest pain. At the age of 3 years, a commissurotomy of the pulmonary valve and infundibulectomy was performed to correct severe infundibular pulmonary stenosis. The initial thoracic CT diagnosed bilateral pulmonary embolism. Echocardiography revealed a filiforme mass in the pulmonary artery 33 mm in length, floating in the right outflow tract, suggesting a pulmonary sarcoma. In addition, a moderate pulmonary valve insufficiency with mild stenosis was diagnosed. Cardiac magnetic resonance (CMR) confirmed the mass in the left pulmonary artery (PA) with floating parts and a small mass in the right PA (Figure 1) and provided complementary findings identifying the mass as tumor, differentiating it from chronic thromboembolism. In particular, perfusion CMR¹ demonstrated blood flow in the mass (Figure 2A and 2B), and late contrast-enhanced CMR revealed high- and low-signal territories in the tumor suggesting heterogenous cell-rich and cell-depleted components of the mass (Figure 3).²

View larger version (96K):   Figure 1. Mass in the left and right PA in axial (A) and sagittal (B) views (steady-state free precession) are shown.

 

View larger version (49K):   Figure 2. Perfusion CMR shows signal increase during first pass of contrast medium through the mass at peak bolus (A) and represented as upslope map and signal-intensity–time curves (B).

 

View larger version (114K):   Figure 3. Late gadolinium-enhanced CMR identifies irregular enhancement patterns in the tumor (the optimal TI was set to null normal myocardium).

 
Histology as shown in Figure 4 showed the mass to be an intimal sarcoma with widespread vascularization with cell-rich and myxoid, cell-depleted . . . [Full Text of this Article]