Background: Primary cardiac neoplasm is rare and generally benign. Epithelioid haemangioendothelioma, a potentially malignant tumour of vascular origin, has been occasionally described in the heart. Composite haemangioendothelioma, characterized by a heterogeneous architecture of vascular components and usually located in soft tissue of the extremities, has only been reported twice in the heart. We herein report another case of this extremely uncommon cardiac tumour.
Case summary: Comprehensive cardiac examination of a 59-year-old female patient with palpitations and personal history of Hodgkin's lymphoma and chest radiation revealed a mass in the left atrium. After surgical resection, histopathological and immunohistochemical analysis identified a composite haemangioendothelioma. After two years, repeated imaging revealed neither signs of local relapse nor metastasis.
Conclusions: Composite haemangioendothelioma, a very uncommon form of potentially malignant vascular tumour, can also be encountered in the heart. In this present case, the outcome was favourable two years after surgical resection without adjuvant therapy.
Keywords: Case report; Composite hemangioendothelioma; Hodgkin’s lymphoma; Primary cardiac neoplasm.
© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.