Low grade myxofibrosarcoma in the right ventricle presenting as pulmonary thromboembolism

Abstract

Abstract:We report an unusual malignant cardiac neoplasm that initially presented as pulmonary thromboembolism in a 78-year-old male. Despite anticoagulation, the pulmonary artery lesion progressed and a mass-like lesion developed in the right ventricular outflow tract. Venoarterial extracorporeal membrane oxygenation was applied before surgery due to the presence of severe right-side heart failure with pulmonary hypertension. A round mass was found in the right ventricle and separate lobulated masses were found in both pulmonary arteries, and these were consistent with low-grade myxofibrosarcoma.

Multiple cardiac myxofibrosarcomas with complete right pulmonary artery occlusion: a case report

“…The lesions are classified as low-grade neoplasms with fibroblastic differentiation and lightly myxoid stroma, endocardial cellular atypia (spindle, round, and oval shaped cells), and nuclear pleomorphism. The low-grade MFS tends to metastasize locally, whereas the high-grade MFS invades local tissue and often metastasizes to distant organs such as the lung, bone, brain, and lymph system ( 9 , 10 ). It was determined that the patient’s cardiac lesions were low-grade MFS.…”

“…Chemotherapy and radiotherapy may be considered in the case of high-grade residual lesions or tumors. Due to the limited number of cases, there is uncertainty about the role of radiotherapy and chemotherapy ( 9 ). Fortunately, our case did not show a local or distant metastasis, and the mass was resected successfully.…”

Intraluminal EWSR1–CREB1 gene rearranged, low‐grade myxoid sarcoma of the pulmonary artery resembling extraskeletal myxoid chondrosarcoma (EMC)

“…Tumours of the pulmonary artery often show prominent or even predominant intraluminal growth in the form of clinically aggressive intimal sarcomas, that are often associated with amplifications of the 12q13‐15 chromosomal region . Low‐grade malignant tumours are less common in these locations and there are few data on their genetic background. In fact, there is only one report of a purely intraluminal primary tumour of a pulmonary artery in a 76‐year‐old woman, showing microscopic features of AFH and carrying EWSR1–ATF1 gene fusion .…”

“…11 However, some such tumours are referred to as of low-grade malignancy. 12,13 Molecular characterisation in these reports is lacking. There is only one report on an intraluminal tumour of pulmonary artery morphologically corresponding to AFH, which was found to contain an EWSR1-ATF1 gene fusion.…”

“…There are rare reports on myxoid tumours in the large vessels or in the heart, mainly corresponding to pleomorphic sarcomas with focal myxoid change . However, some such tumours are referred to as of low‐grade malignancy . Molecular characterisation in these reports is lacking.…”