Primary cardiac neoplasms are very uncommon; the autopsy incidence is 0.0001–0.03% [1]. Sarcomas are the most common primary cardiac malignancies, with angiosarcoma being the most common histological subtype [1]. Cardiac angiosarcoma is very aggressive with rapid progression. Here, we report a case of primary cardiac angiosarcoma with lung metastases compli-cated by massive hemothorax in a 34-year-old male.
CASE REPORT
A 34-year-old male presented with syncope and serial echo-cardiograms showing recurrent pericardial effusions. Pericar-diocentesis yielded blood-stained pericardial fluid with nega-tive cytology. Serum parvovirus B19 PCR was positive, and he was given a course of intravenous immunoglobulin. However, interval development of a right atrial echogenic mass was not-ed (Fig. 1A), and he was put on anticoagulation treatment in view of the suspicious right atrial thrombus. Repeat echocar-
diogram showed a grossly dilated right atrium with enlargement of the echogenic mass infiltrating the tricuspid valve annulus and right ventricular free wall (Fig. 1B). A subsequent MRI re-vealed a grossly enlarged and akinetic right atrium with atrial wall thickening, evidence of thrombi, and pseudoaneurysm formation (Fig. 1C, Supplementary Video 1 in the online-only Data Supplement). The right atrial wall mass showed evidence of first-pass perfusion and enhancement (Fig. 1D, Supplemen-tary Video 2 in the online-only Data Supplement). In addition, pericardial invasion and compression of the right ventricular free wall were observed (Fig. 1E). Bilateral lung nodules were suggestive of lung metastases (Fig. 1F). CT-guided core needle biopsy of the dominant lung nodule was performed, and micro-scopic examination showed cellular spindle cell proliferation forming ill-defined slit-like channels (Fig. 2A). Immunohisto-chemical staining showed strong positivity for endothelial mark-ers CD31 (Fig. 2B) and ERG (Fig. 2C), consistent with angiosar-coma. During his hospital stay, CT showed progressive dilatation of the right atrial pseudoaneurysm (Fig. 3A) and active contrast extravasation from the tumor, resulting in large hemothorax (Fig. 3B). Interval progression of lung metastases was also noted (Fig. 3C). The patient was put on pleural drainage and received paclitaxel and pazopanib. He finally succumbed to the disease
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CVIA Cardiac Angiosarcoma Heralded by Recurrent Hemorrhagic Pericardial Effusions–Utility of MRI and CTFiona Fong-ying Wan1, Jonan Chun-yin Lee1, Jeanie Betsy Chiang1, Kim-hung Tsang1, Cheuk-bong Ho2, Eric Chi-yuen Wong2
1 Departments of Radiology & Imaging, 2Medicine, Queen Elizabeth Hospital, Kowloon, Hong Kong
Received: October 18, 2019Revised: December 17, 2019Accepted: December 20, 2019
Corresponding authorFiona Fong-ying Wan, MBChBDepartment of Radiology & Imaging, Queen Elizabeth Hospital, 30 Gascoigne Road, Kowloon, Hong KongTel: 852-35062881Fax: 852-35066048E-mail: [email protected]
Cardiac angiosarcoma is a rare primary cardiac malignancy that should be considered in pa-tients with recurrent pericardial effusions, especially those with spontaneous hemorrhagic pericardial effusion. A 34-year-old man presented for evaluation of pericardial effusion. Initial imaging and pericardial histopathological workup failed to provide a definitive diagnosis. Se-rum parvovirus B19 PCR test was positive and he was treated for viral pericarditis. In view of his recurrent hemorrhagic pericardial effusions and persistent right atrial thrombus, MRI was performed and revealed findings suggestive of cardiac angiosarcoma with lung metastases. The diagnosis was confirmed histologically. The patient’s condition progressed rapidly with massive hemothorax, and although he received systemic therapy for disease control, he suc-cumbed to the disease five months after diagnosis confirmation.
Key words Cardiac tumor · Angiosarcoma · Magnetic resonance imaging · Pericardial effusion.
pISSN 2508-707X / eISSN 2508-7088
Cardiovasc Imaging Asia 2020;4(1):30-33https://doi.org/10.22468/cvia.2019.00164
Fiona Fong-ying Wan, et al CVIAfive months after diagnosis confirmation.
DISCUSSION
Cardiac angiosarcoma is an extremely rare aggressive tumor. It typically occurs in the third to fifth decades of life, with a male predominance [1]. It usually develops in the right atrium with invasion of adjacent structures and frequent pericardial involve-
ment. Therefore, patients typically present with right sided heart failure or tamponade, often with superimposed systemic signs such as fever and weight loss [2].
Echocardiography, usually the initial imaging examination, is limited by its trans-thoracic window and inability to adequate-ly characterize different tissue types. CT is particularly useful in detecting complications, including pseudoaneurysm formation and active tumor bleeding. However, CT assessment of a cardi-
A
D E F
B C
Fig. 1. Right atrial malignant cardiac tumor with lung metastases. A: Trans-esophageal echocardiogram (TEE) showed a right atrial echogenic mass suspicious for thrombus (arrow). B: TEE later showed dilated right atrium with interval enlargement of the heterogeneous echogenic mass over the posterior right atrium (arrow). C: T2 turbo spin-echo (TSE) sequence showed pseudoaneurysm formation of the right atrium, with T2W hyperintense right atrial wall mass and T2W hypointense lesions, representing thrombi. D: Heterogeneous enhancement of the right atrial mass was noted. The right atrial thrombus did not enhance. A right lung nodule was present (arrow). E: Late post-gadolinium T1 TSE se-quence showed pericardial invasion and compression on the right ventricular free wall. F: Bilateral lung metastases were noted.
A B CFig. 2. Microscopic examination of the lung biopsy demonstrated cellular spindle cell proliferation forming ill-defined slit-like channels, with spindle cells possessing hyperchromatic pleomorphic nuclei (A) (hematoxylin and eosin staining, original magnification ×400). Immunohisto-chemical staining of the spindle cells revealed strong positivity for the endothelial markers CD31 (B) (original magnification ×400) and ERG (C) (original magnification ×400).
32 Cardiovasc Imaging Asia 2020;4(1):30-33
Imaging Findings of Cardiac AngiosarcomaCVIA
ac tumor is somewhat limited. The tumor can be misinterpreted as a thrombus, which may co-exist, as in our case. A cardiac tu-mor may be overlooked on enhanced CT scan since its hyper-vascularity can appear very similar in attenuation to blood pool [3]. Therefore, the presence of recurrent pericardial effusions, spontaneous hemorrhagic pericardial effusion, or right atrial thrombus should prompt vigilant inspection for this entity and further investigation with MRI.
MRI is particularly useful in differentiating between throm-bus and neoplasia by delayed enhancement with gadolinium. Cardiac tumors show increased vascularity with contrast en-hancement, whereas thrombi remain dark [4]. On MRI, cardi-ac angiosarcoma appears heterogeneous due to presence of in-tralesional necrosis and hemorrhage, and it is predominantly T1-weighted isointense and T2-weighted hyperintense to the myocardium. T1- and T2-weighted turbo spin-echo “dark-blood” sequences are particularly helpful in visualization of the tumor, as the signal intensity of the tumor may be similar to that of adjacent blood pool on “bright-blood” sequences. Flow voids can occasionally be identified within the mass due to extensive vascularity. Tumor infiltration into adjacent structures, includ-ing pericardium, inter-atrial septum, and superior or inferior vena cava, is common. Hemorrhagic pericardial effusion and hemothorax are possible complications. Necrotic impairment of the heart wall can even result in myocardial rupture [5].
The differential diagnoses of right atrial mass include benign entities such as myxoma and thrombus as well as malignant pa-thologies such as metastasis, primary cardiac lymphoma and primary cardiac angiosarcoma [6]. The combination of clinical history, disease progression and imaging is essential for diagno-sis confirmation. Unlike benign entities, cardiac angiosarcoma presents with rapid disease progression. Pseudoaneurysm for-mation, as in our case, is a key imaging feature to distinguish an-giosarcoma from other malignant pathologies. When lung me-tastases are present, a distinctive halo of ground glass change around nodules representing peri-nodular hemorrhage into al-veoli can sometimes be identified [7].
Histological diagnosis is required for definitive determination of tumor type and accurate therapy planning. However, cytol-ogy of pericardial puncture often fails to yield malignant cells, even when pericardial invasion is present [4]. Endomyocardial biopsy is non-diagnostic in most cases and carries a high risk of bleeding. Therefore, a meticulous diagnostic workup with mul-timodality imaging is essential.
There is currently no standardization in therapeutic options, given the rarity of this disease. Radical surgical removal of the tumor, although almost always impossible due to the extent of local invasion or presence of distant metastases, is considered the most effective treatment. Therefore, a multimodality ap-proach combining surgery with radiotherapy and chemothera-py is used most often. Even with treatment, there is a high rate of local recurrence and systemic metastases. The prognosis is poor, and most patients succumb to the disease within months of diagnosis [1].
In summary, cardiac angiosarcoma is a rare but aggressive disease with rapid progression. Clinicians and radiologists should keep this rare disease in mind for patients with recurrent peri-cardial effusions of unknown etiology, spontaneous hemor-rhagic pericardial effusion, or right atrial thrombus.
Supplementary Video LegendsVideo 1. Steady-state free precession (SSFP) cine images showed akinetic
and grossly dilated right atrium with pseudoaneurysm formation.Video 2. First-pass dynamic sequence showed perfusion of the right atrial
posterior wall mass.
Supplementary MaterialsThe online-only Data Supplement is available with this article at https://
doi.org/10.22468/cvia.2019.00164.
Conflicts of InterestThe authors have no potential conflicts of interest to disclose.
AcknowledgmentsAuthor appreciation goes to Pathologist Dr. Angie Kwok and Department
of Pathology from Queen Elizabeth Hospital. Their contributions are grate-fully acknowledged.
A B CFig. 3. Contrast CT showed progression of the disease complicated by hemothorax. A: Cardiac angiosarcoma caused progressive dilatation of a right atrial pseudoaneurysm. B: Cardiac angiosarcoma complicated by large right hemothorax with active bleeding. C: Interval progres-sion of lung metastases was noted.
www.e-cvia.org 33
Fiona Fong-ying Wan, et al CVIAORCID iDs
Fiona Fong-ying Wan https://orcid.org/0000-0002-3897-3348Jonan Chun-yin Lee https://orcid.org/0000-0002-9157-6982Jeanie Betsy Chiang https://orcid.org/0000-0002-8364-4500Kim-hung Tsang https://orcid.org/0000-0002-3994-1188Cheuk-bong Ho https://orcid.org/0000-0001-9481-6390Eric Chi-yuen Wong https://orcid.org/0000-0002-5030-3216
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