Giant Pericardial Hemangioma with a Large Central Fibrotic Core

Pericardial hemangioma is a very rare cardiac tumor that has a relatively desirable prognosis if properly treated. Correct diagnosis and good surgical planning, which can be provided by multimodality imaging, are important parts of successful treatment. We presented a case of a middle-aged man with a pericardial mass that displayed typical findings of hemangioma in various imagings. This case highlighted the essential of multimodality imaging, particularly computerized tomography (CT) and cardiac magnetic resonance imaging (CMR), to make a correct diagnosis leading to definite treatment. Pericardial hemangioma is a very rare cardiac tumor that has a relatively desirable prognosis if properly treated. Correct diagnosis and good surgical planning, which can be provided by multimodality imaging, are important parts of successful treatment. We presented a case of a middle-aged man with a pericardial mass that displayed typical findings of hemangioma in various imagings. This case highlighted the essential of multimodality imaging, particularly computerized tomography (CT) and cardiac magnetic resonance imaging (CMR), to make a correct diagnosis leading to definite treatment. A 53-year-old previously healthy man presented with progressive dyspnea for four months. Physical examinations including cardiopulmonary examination were normal. Laboratory investigations and electrocardiography yielded unremarkable results. Plain chest radiography depicted a mass-like opacity in the left lower lung zone silhouetted with a left cardiac border. (Figure 1A ) Transthoracic echocardiography showed a large echogenic pericardial mass with a central hypoechoic core adjacent to the anterolateral wall of the left ventricle. (Figure 1B) There was no hemodynamic consequence to blood flow in the heart based on Doppler echocardiography. CT of the chest demonstrated an 11.8 x 8.5 x 10.1 cm isodense mass (42 Hounsfield units at the periphery) with a large internal hypodense portion (19 Hounsfield units at the central core) and partial peripheral nodular enhancement at the left-sided pericardium. (Figure 1C and Supplemental Figure S1) The provisional diagnoses were angiosarcoma, hemangioma, and other vascularized tumors. Since this mass caused the symptom and malignancy could not be excluded, the patient was advised and informed consent by the heart team to undergo sternotomy with pericardial mass removal. Unfortunately, the first operation failed since the surgeon was not confident enough to en bloc resection mass that appeared to adhere to the left atrium in the operative field. Moreover, the benignity of the mass was still uncertain. Coronary angiography and CMR were prescribed to characterize the mass further. Coronary angiography illustrated normal coronary arteries with evidence of a feeding vessel from the left anterior descending artery to the mass and tumor blush. (Figure 1D) Regarding CMR, the mass was well-circumscribed with an iso-intensity on T1-weighted and hyper-intensity on T2-weighted images. (Figures 1E-1F) First-pass perfusion images depicted a focal rapid enhancement at the periphery of the mass, corresponding with the evidence of a feeding vessel from coronary angiography. (Supplemental Figure S2A) The periphery of the mass was homogeneously enhanced in late gadolinium enhancement images, while the central core was not. (Figure 1G) Pre-contrast parametric mappings demonstrated high T1 and T2 values of the mass (equal to the blood in the cardiac chamber) with the highest at the central core. (Figures 1H and Supplemental Figure S2B) Based on CMR findings, pericardial hemangioma and angiosarcoma were the most and second-most likely diagnoses, respectively. After confirming that the mass had not infiltrated the adjacent organs, the second operation was performed under meticulous planning and successfully excised the entire mass without complication. The mass was rubbery consistency and appeared well-circumscribed, dark-brown color with a yellowish central core. (Figures 3A-3C) Histology from the periphery of the mass showed numerous irregular blood-filled spaces with endothelial lining and dense fibrotic tissue surrounding the spaces, consistent with cavernous hemangioma. (Figure 3D) The central core was composed of loose fibrotic tissues. (Figure 3E) The postoperative period was uneventful. The patient was discharged on the 7th day after the operation. At the 3-month follow-up visit, the patient was doing well and could perform an exercise without any restriction.Figure 2Gross pathology demonstrated a well-circumscribed mass with a dark-brown color and a yellowish central core (A-C). Histology from the periphery of the mass showed numerous irregular blood-filled spaces with endothelial lining and dense fibrotic tissue surrounding the spaces, consistent with cavernous hemangioma (D). The central core was composed of loose fibrotic tissues (eosinophilic linear structures) (E).View Large Image Figure ViewerDownload Hi-res image Download (PPT) Cardiac hemangioma is a rare cardiac tumor accounting for 2.8% of primary cardiac tumors. (1Li W. Teng P. Xu H. Ma L. Ni Y. Cardiac Hemangioma: A Comprehensive Analysis of 200 Cases.Ann Thorac Surg. 2015; 99: 2246-2252Abstract Full Text Full Text PDF PubMed Scopus (74) Google Scholar) Pericardial hemangioma is the rarest subtype with less than 20 case reports to date. (2Seitz A. Ong P. Backes M. Mahrholdt H. Chronic pericardial effusion in the setting of pericardial capillary haemangioma: a case report and review of the literature.Eur Heart J Case Rep. 2018; 2yty024PubMed Google Scholar) Clinical manifestations of pericardial hemangioma can be varied from asymptomatic to life-threatening conditions depending on the size and location of the mass. Dyspnea and pericardial effusion are common presentations. (2Seitz A. Ong P. Backes M. Mahrholdt H. Chronic pericardial effusion in the setting of pericardial capillary haemangioma: a case report and review of the literature.Eur Heart J Case Rep. 2018; 2yty024PubMed Google Scholar) Multimodality imaging is necessary to make an accurate diagnosis. Plain chest radiography is acquired in most cases as a routine investigation because of its accessibility and noninvasiveness; however, it cannot diagnose the type of mass and may sometimes overlook masses with a small size. Echocardiography is considered the first-line modality since it can assess the mass size, location, and effect on cardiac structures and functions including hemodynamic consequences. Given the advancement of noninvasive imaging modalities, coronary angiography is rarely utilized unless the coronary artery system needs to be evaluated. Feeding vessels and tumor blushing (cotton wool or snowy tree appearance) may be revealed after contrast injection. (3Johnson C.M. Sheedy 2nd, P.F. Stanson A.W. Stephens D.H. Hattery R.R. Adson M.A. Computed tomography and angiography of cavernous hemangiomas of the liver.Radiology. 1981; 138: 115-121Crossref PubMed Scopus (83) Google Scholar) To narrow down or make the diagnosis of mass subtypes, CT and CMR are the most useful modalities since hemangioma has typical findings in these modalities. (4Klotz T. Montoriol P.F. Da Ines D. Petitcolin V. Joubert-Zakeyh J. Garcier J.M. Hepatic haemangioma: common and uncommon imaging features.Diagn Interv Imaging. 2013; 94: 849-859Crossref PubMed Scopus (78) Google Scholar) In addition, both CT and CMR are crucial for planning the operative strategy. The characteristics of cardiac hemangioma, including pericardial, are similar to hemangioma in other locations. Hemangioma appears well-circumscribed and isodense with a density (and also the Hounsfield units) equal to blood in non-contrast CT and becomes enhanced with peripheral nodular and centripetal filling patterns after contrast administration. (4Klotz T. Montoriol P.F. Da Ines D. Petitcolin V. Joubert-Zakeyh J. Garcier J.M. Hepatic haemangioma: common and uncommon imaging features.Diagn Interv Imaging. 2013; 94: 849-859Crossref PubMed Scopus (78) Google Scholar) In CMR, hemangioma classically displays hypo- to iso-intensity on T1-weighted and hyper-intensity on T2-weighted images. (5Semelka R.C. Brown E.D. Ascher S.M. et al.Hepatic hemangiomas: a multi-institutional study of appearance on T2-weighted and serial gadolinium-enhanced gradient-echo MR images.Radiology. 1994; 192: 401-406Crossref PubMed Google Scholar) Parametric mappings, including native T1 and T2 mappings, directly indicate T1 and T2 relaxation times enhancing the tissue characterization property of CMR and can help differentiate the mass types. Masses composed of fat (lipoma, liposarcoma), methemoglobin (recent hemorrhage), and melanin (melanoma) have a low T1 relaxation time while masses composed of fibrosis (fibroadenoma, fibrotic core) and blood (hemangioma, angiosarcoma) have a high T1 relaxation time. T2 relaxation time is high in fluid-filled cystic lesions and hypervascularized tumors (e.g., hemangioma, angiosarcoma). (6Bonnes J. Brink M. Nijveldt R. How to evaluate cardiac masses by cardiovascular magnetic resonance parametric mapping?.Eur Heart J Cardiovasc Imaging. 2023; 24: 1605-1607Crossref PubMed Scopus (2) Google Scholar) Three patterns of enhancement were described in hepatic hemangioma which may be applied to cardiac hemangioma: (i) rapid homogeneous enhancement on first-pass perfusion images (also known as flash-filling hemangioma which is typically found in small hemangioma), (ii) peripheral nodular enhancement with progressive centripetal filling to homogeneously enhancement (most common patterns), and (iii) peripheral nodular enhancement with persistent centrally unenhanced (typically found in large hemangioma, size > 5 cm). (5Semelka R.C. Brown E.D. Ascher S.M. et al.Hepatic hemangiomas: a multi-institutional study of appearance on T2-weighted and serial gadolinium-enhanced gradient-echo MR images.Radiology. 1994; 192: 401-406Crossref PubMed Google Scholar) Unlike hepatic hemangioma, an enhancement with central sparing is rarely reported in cardiac hemangioma. To our knowledge, our case is the second case of pericardial hemangioma that exhibited this enhancing pattern. (7Ben Youssef A. Zairi S. Ouerghi S. et al.Epicardial cavernous hemangioma: a two-case report.Tunis Med. 2014; 92: 268-271PubMed Google Scholar) An unenhanced central core is an area of scarring and fibrosis usually formed within a large mass of more than 3-5 cm due to insufficient blood supply. (5Semelka R.C. Brown E.D. Ascher S.M. et al.Hepatic hemangiomas: a multi-institutional study of appearance on T2-weighted and serial gadolinium-enhanced gradient-echo MR images.Radiology. 1994; 192: 401-406Crossref PubMed Google Scholar) Since pericardial and mediastinal spaces are smaller than intra-abdominal space, patients with pericardial hemangioma generally develop symptoms earlier and at a smaller size of mass. The definite treatment of pericardial hemangioma is to complete mass removal, particularly when symptoms occur. Our case nicely demonstrated classic image findings of pericardial hemangioma and emphasized the essential of multimodality imaging, particularly CT and CMR, to make a correct diagnosis leading to definite treatment. -Pericardial hemangioma is a very rare cardiac tumor that has a relatively desirable prognosis if properly treated.-Multimodality imaging, particularly CT and CMR, can provide a correct diagnosis and good surgical planning.-Peripheral enhancement with central sparing is typically found in a large hemangioma with a size of > 3-5 cm and rarely reported in cardiac hemangioma. This may be due to the smaller size of mass at the presentation compared to hepatic hemangioma. This is a retrospective case report using de-identified data; therefore, the IRB did not require consent from the patient. No funding was provided for this paper.