Abstract 17111: Spontaneous Regression of Cardiac Angiosarcoma in a 17-Year-Old Male

Abstract

Description of Case: A fit and healthy 17-year-old male was admitted with a 10-day history of chest pain and SOB. He was taking no medication and had no significant past medical or family history. Clinical examination revealed signs of pericarditis with widespread ST elevation and PR depression on ECG. TTE showed a moderate, global pericardial effusion. He was treated for pericarditis with anti-inflammatory medication and discharged with follow up a few weeks later. Repeat TTE demonstrated a mass in the pericardial space adjacent to the RV free wall. Urgent CMR was requested to exclude malignancy and showed a large, irregular heterogenous mass adjacent to the RA and RV with invasion into the RV free wall. CMR characterisation including first pass perfusion was indeterminate. CT TAP showed no distant metastases. Percutaneous biopsy was non diagnostic and serial CMR 3 weeks later showed significant reduction of the mass. PET-CT confirmed reduction in the size of the mass although there was moderate heterogeneous FDG uptake, more suggestive of malignancy rather than a benign lesion. Given the apparent resorption of the mass, repeat CMR was planned 3 months later. Repeat CMR (6 months after initial CMR) showed significant increase in the size of the cardiac mass. Surgical biopsy confirmed cardiac angiosarcoma and was unresectable. After MDT discussion, the patient was started on doxorubicin, dexrazoxane and pazopanib. This has failed to control the tumour, and he has now commenced a trial of paclitaxel. Discussion: Spontaneous regression of cardiac rhabdomyoma in infants has been described, but this does not extend to other primary cardiac tumours or adults. The re-emergence and significant increase in size of the mass suggests initial regression may have been a percutaneous biopsy effect causing collapse or resorption of the necrotic core. Suggested mechanisms include disruption of the tumour feeding artery during biopsy or activation of the immune system.