Perigraft hygroma mimicking recurrent angiosarcoma of the right atrium

Abstract

Primary cardiac angiosarcoma is extremely rare, has a poor prognosis and has no specific clinical manifestation. A 31-yearold pregnant woman presented to the emergency department with signs of pericardial tamponade. Transesophageal echocardiography (TEE) showed a mass within her right atrium. Extensive surgical resection was performed with subsequent pericardial patch reconstruction of the right atrium. Histological examination showed the presence of an angiosarcoma. A reoperation was performed 18 months after the initial operation, because of concerns of radicality. Three years later she was re-admitted with a superior caval vein syndrome. A stent was placed to relieve symptoms. Because of the suspicion of a recurrent tumor, a second reoperation followed. During this operation, a perigraft hygroma was found pressing against the previously reconstructed superior caval vein. Histological examination after both reoperations showed no signs of a recurrent angiosarcoma. Until today, 8 years after her first surgery, the patient is still alive without any signs of recurrent tumor.

Incidental cardiac lymphangioma misdiagnosed as atrial thrombus: A case report

“…Asymptomatic lymphangiomas, however, are not uncommon and are therefore found only incidentally [ 2 , 3 ]. Association of cardiac lymphangiomas with other lesions, including breast cancer, anatomically distinct lymphangioma, and cardiac angiosarcoma, is well-described, and may facilitate the detection of asymptomatic cardiac lymphangiomas [ 1 , 10 ]. Conversely, laboratory investigations may display nonspecific disturbances such as leukocytosis, elevated erythrocyte sedimentation rate or C-reactive protein, thrombocytopenia, or hemolytic anemia, which are present in a host of other conditions and might complicate differential diagnoses [11] .…”

“…While cardiac lymphangiomas may vary in their clinical presentations, almost all of the reported cases have been uniformly treated with open surgical tumor resection [1] . In spite of the benign nature of cardiac lymphangiomas, fear of possible cardiac and hemodynamic complications, or lack of preoperative histopathological characterization of the lesion may justify the clinical inclination for surgical treatment [ 10 , 12 ]. Conversely, treatment of other primary cardiac tumors is based on their symptomatology and histopathological nature, which could mandate unnecessary surgical interventions [6] .…”

“…We conducted a PubMed search for cardiac lymphangioma cases being reported between 2018 and 2022 using the MeSH terms “heart” and “lymphangioma.” Only one case of cardiac lymphangioma was reported in the respective timeframe; Ko et al described a variant of cardiac lymphangioma in the form of hygroma at the superior vena cava opening. A cardiac lesion causing superior vena cava syndrome in a patient with a history of cardiac angiosarcoma, was surgically excised in case of malignant recurrence, which turned out to be hygroma [10] .…”