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Image of the Month: Unusual sinusoid like formations in the septum of a young lady

On the left: Masson Trichrome staining                    
On the right: SM-Aktin(brown) staining for myofibroblasts  

A 12 year old girl presented with a systolic murmur when she was routinely investigated by the school doctor. For evaluating this murmur she underwent echocardiography. Here, a cardiac tumor was identified in the interventricular septum resulting in a mainly right ventricular outflow tract obstruction. The removal of the tumor (3x 2x 2 cm) by a heart surgeon was without any complications. Histopathological investigations of the tissue revealed a considerable loss of cardiomyocytes in association with a severe fibrosis (blue tissue in Masson Trichrome staining). We observed an unusual benign sinusoidal formation of structures which were CD34 negative but associated with large, focal aggregates of SM- Aktin positive myofibroblasts as demonstrated by immunohistochemistry in the right image. So far, the origin of this structural aberrance is totally unclear.  

The content of this article reflects the personal opinion of the author/s and is not necessarily the official position of the European Society of Cardiology.