A case of rapid progression of a cardiac tumor originating from the coronary sinus observed by transthoracic echocardiog

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IMAGE–CARDIOLOGY

A case of rapid progression of a cardiac tumor originating from the coronary sinus observed by transthoracic echocardiography Toshimitsu Kato1   · Noriaki Takama2 · Yohei Ishibashi2 · Tomonari Harada2 · Masaru Obokata2 · Masahiko Kurabayashi2 · Masami Murakami1 Received: 11 May 2020 / Accepted: 29 August 2020 © The Japan Society of Ultrasonics in Medicine 2020

A 75-year-old woman presented with shortness of breath. Her lactate dehydrogenase (298 U/L), D-dimer (3.1 μg/ mL), and brain natriuretic peptide (69.6 pg/mL) levels were slightly elevated. Electrocardiogram showed normal sinus rhythm. Chest X-ray showed a cardiothoracic ratio of 68%. The lung field was clear. Transthoracic echocardiography (TTE) was performed. The left ventricular contractility was preserved (ejection fraction 76%). However, a low-isoechoic tumor was observed posterior and lateral between the left atrium (LA) and left ventricle (LV) (Fig. 1a–c). The inside of the tumor was inhomogeneous with no color Doppler signal. Coronary angiography revealed feeding arteries to the tumor (Fig. 1d, e). Contrast chest computed tomography (CT) revealed a non-uniform low-contrast tumor adjacent to the LA and LV (Fig. 1f). The tumor showed an abnormal maximum standardized uptake value (SUVmax) (2.7–6.0) on fluorodeoxyglucose-positron emission tomography (FDG-PET) (Fig. 1g). Biopsy was not performed considering the high risk of fatal bleeding. Neither surgical resection, radiotherapy, nor chemotherapy was indicated. Two months later, her shortness of breath exacerbated. TTE showed enlargement of the tumor, increase of pericardial fluid, and projection of the tumor to the right atrium (RA) (Fig. 1h, i). CT revealed projection of the tumor to the RA and pulmonary artery embolism (Fig. 1j, k).

Cardiac tumor uptake of SUVmax > 3.5 on FDG-PET indicates malignancy [1]. In this case, there were no findings suggesting metastasis. The tumor was suspected to be primary cardiac malignant tumor (SUVmax 6.0 > 3.5). The frequencies of primary cardiac malignant tumors are as follows: cardiac angiosarcoma (CA), 45%; fibroblastic cardiac sarcoma, 18%; pericardial tumor, 14%; cardiac rhabdomyosarcoma, 9%; primary cardiac lymphoma, 9%; and cardiac leiomyosarcoma, 5% [2]. A homogeneous internal echo of a primary cardiac lymphoma reflects less necrosis. However, an inhomogeneous internal echo (like this case) is characteristic of a CA, reflecting partial bleeding and necrosis [3]. Primary cardiac fibroblastic sarcomas and myosarcomas originate from the LV. However, CAs originate from the right side of the heart. CAs rapidly progress, and metastasis to the lung frequently occurs [5]. In this case, the tumor originated from and rapidly extended along the coronary sinus and projected to the RA, which was presumably associated with pulmonary artery tumor embolism or metastasis. The tumor was clinically assumed to be a CA. The patient’s prognosis was predicted to be extremely poor. The rapid clinical course of a CA originating from the coronary sinus was observed usi