Pathology:
Histopathological examination of resected mass showed malignant mesenchymal neoplasm composed of sheets and individual tumoral cells embedded in a desmoplastic and collagenized stroma. These cells are charactherized by enlarged, pleomorphic hyperchromatic and vesicular nuclei with frequent mitotic figures and visible nucleoli. Necrosis and hemorrhage are also present. Immunohistochemistry staining were negative for S100 and CD34 but positive for vimentin, desmin, smooth muscle actin (SMA), MNF116. In addition, about 15-20% with Ki67 also positive in osteoclast like giant cells with CD68. (Figure 7) All this data shows malignant mesenchymal neoplasm compatible with undifferentiated pleomorphic sarcoma.
The patient referred to oncologist for chemotherapy. Just the day before initiation of chemotherapy, TTE was done and recurrence of tumoral mass (1.5*1.5 cm) was seen in distal part of RVOT just beneath PV. Patient admitted in hospital for receiving Chemotherapy protocol with carboplatin and taxotere (docetaxel) with close monitoring of cardiac condition and hemodynamic. Unfortunately, after one course of chemotherapy patient was died in hospital with cardiogenic shock because of severe right sided heart failure.