First Paediatric case of Cardiac composite Hemangioendothelioma

Abstract Background Primary cardiac tumours in children are exceptionally rare. Majority are benign, with only about 10% being malignant; rhabdomyosarcoma is reported to be the commonest variety . We present the first paediatric case of a rare vascular tumour in an extremely rare location (right atrium) hitherto unreported in literature. Childhood cardiac tumour may go unnoticed, so we need to know its presentations, age groups involved, correlative radiological findings and histology. Confirmation is based on the histological distinction of various closely related cardiac tumours. Diagnostic dilemmas arise in differentiating cardiac angiosarcoma from composite hemangioendothelioma as in our case. Accurate identification of primary pathology is imperative for guiding management and prognostication. Case Report A 12-year-old girl presented with cough, abdominal pain, dizziness, and loss of consciousness. On evaluation, tumour in right atrium was identified along with massive haemorrhagic pericardial and bilateral pleural effusion raising the suspicion of rhabdomyosarcoma. Salient imaging findings in this case include a large right atrial mass with massive pericardial effusion and cardiac tamponade on echocardiography. Contrast enhanced computed tomography (CECT) provided better characterisation of the primary mass lesion with minimal post-contrast enhancement. Cardiac magnetic resonance imaging (CMR) additionally revealed accurate localisation of mass along right atrial free wall an associated large thrombotic component; areas of subtle hyper vascularity, STIR hyper intensity and LGE (late gadolinium enhancement) within the lesion. Histology and double staining immunohistochemistry (IHC) revealed a composite hemangioendothelioma of cardiac origin (HE). Patient underwent excision of myocardial mass and has recently completed the first cycle of chemotherapy. Conclusion Differentiating Cardiac HE from angiosarcoma is imperative given the poor prognosis of angiosarcomas and better outcomes in the former. CMR was helpful in aiding the diagnosis of such rare and remotely located tumours. In general, the prognosis in HE is reported as favourable, with a 50% risk of local recurrence and a low risk of lymph nodal or distant metastases. This case highlights that all paediatric tumours however rare must undergo all battery of investigations so that one can achieve the highest clinical outcome.