Giant cavernous hemangioma of the left atrial appendage: a case description and literature review
Letter to the Editor

Giant cavernous hemangioma of the left atrial appendage: a case description and literature review

Qing Duan1#, Hongwei Li2#, Gaowu Yan1#, Yang Yang1, Jiantao Deng1, Ling Yang3, Morgan A. McClure4, Anup Bhetuwal5, Jing Yan1#, Guoqing Yang1*, Yong Li1, Linwei Zhao1, Xiaoping Fan1

1Department of Radiology, Suining Central Hospital, Suining, China; 2Department of Radiology, The Third Hospital of Mianyang, Sichuan Mental Health Center, Mianyang, China; 3Department of Pathology, Suining Central Hospital, Suining, China; 4Department of Radiology and Imaging, Institute of Rehabilitation and Development of Brain Function, The Second Clinical Medical College of North Sichuan Medical College, Nanchong Central Hospital, Nanchong, China; 5Sichuan Key Laboratory of Medical Imaging, Department of Radiology, Affiliated Hospital of North Sichuan Medical College, Nanchong, China

#These authors contributed equally to this work and should be considered as co-first authors.

*These authors contributed equally to this work and should be considered as co-corresponding authors.

Correspondence to: Guoqing Yang; Gaowu Yan. Department of Radiology, Suining Central Hospital, 127 West Desheng Road, Suining 629000, China. Email: snygq@163.com; yangaowu1989@163.com.

Submitted May 07, 2022. Accepted for publication Sep 23, 2022. Published online Oct 18 2022.

doi: 10.21037/qims-22-460

Introduction

In 2015, the World Health Organization (WHO) proposed a new histological classification of cardiac tumors. Cardiac tumors are divided into the following: (I) benign tumors and tumor-like conditions, (II) tumors of uncertain biological behavior, (III) germ cell tumors, (IV) malignant tumors, and (V) tumors of the pericardium (1,2). Clinically, primary cardiac tumors (PCTs) are rare entities, and cardiac hemangiomas (CHs) are particularly rare benign vascular lesions, accounting for approximately 5–10% of benign cardiac tumors and less than 2% of all heart neoplasms (3,4). CHs occur as a result of abnormal dilation or hyperplasia of small arteries, veins, or capillaries (5), and are histologically divided into capillary hemangioma, cavernous hemangioma, and arteriovenous malformation (1,2).

In children, the most common site of CHs is the right atrium (RA), whereas in adults, they are mostly located in the left ventricle (LV) and cardiac valves (1). Cavernous hemangioma of the left atrial appendage is especially rare. Herein, we report a case of cavernous hemangioma of the left atrial appendage. We also investigated the recent correlative literature to summarize its clinical characteristics, imaging manifestations, histopathology, and therapeutic methods.

Case presentation

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was provided by the patient for the publication of this case report and the accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

A 60-year-old woman was admitted with a mediastinal mass that was discovered incidentally on 24 December 2019. In the course of the disease, she had no known history of nausea, vomiting, palpitations, or dyspnea. After admission to the hospital, physical examination revealed normal cardiac sounds with no murmur. Laboratory examination results revealed that all the parameters were within the normal reference range. It was noted that no abnormalities were illustrated on the echocardiogram. Contrast-enhanced computed tomography (CECT) of the chest showed a heterogeneous and well-defined lesion on the left side of the mediastinum measuring 7.0×8.5×6.0 cm (Figure 1A). The mass was well demarcated from the left atrium (LA), LV, and pulmonary artery. In addition, this mass showed nodular and patchy enhancement in the arterial phase (Figure 1B), and the range of enhancement increased in the venous phase (Figure 1C,1D). Our radiologists tried to perform cardiac magnetic resonance (CMR) imaging on this patient to gain a comprehensive understanding of the lesion. However, the CMR examination was unsuccessful because of the patient’s claustrophobia.

Figure 1 A 60-year-old woman was diagnosed with a cavernous hemangioma of the left atrial appendage. (A) On NECT, the mass is on the left side of the patient’s heart (red star). (B-D) At the arterial (B) and venous phases (C,D) of the CECT, it shows a pattern of “progressive” or “prolonged” enhancement (yellow arrows), which are typical findings of a hemangioma. NECT, non-contrast-enhanced computed tomography; CECT, contrast-enhanced computed tomography.

Subsequently, surgical excision of the lesion using a midsternal thoracotomy was scheduled for both definitive diagnosis and therapeutic purposes (Figure 2A,2B). Histopathological examination of the mass confirmed the diagnosis of cavernous hemangioma originating from the left atrial appendage (Figure 3). At 6 months after the surgery, non-contrast-enhanced computed tomography (NECT) examination depicted the absence of the tumor (Figure 4). After discharge, the patient was followed up by an echocardiogram, which showed no recurrence of this tumor.

Figure 2 A 60-year-old woman was diagnosed with a cavernous hemangioma of the left atrial appendage. (A) The lesion attached to the left atrial appendage with a pedicle (white arrow). (B) Gross view of the tumor after resection.
Figure 3 A 60-year-old woman with cavernous hemangioma of the left atrial appendage. Dilated vascular cavities with a large number of red blood cells in the lumen. These cavities have thick walls (HE, ×200). HE, hematoxylin and eosin.
Figure 4 A 60-year-old woman was diagnosed with a cavernous hemangioma of the left atrial appendage. (A,B) Six months after surgery, the NECT examination depicted the absence of the tumor. NECT, non-contrast-enhanced computed tomography.

Discussion

Clinical features

CHs are rare benign vascular tumors originating from the heart, whereas myxomas and papillary fibroelastomas are more common in clinical diagnosis. CHs can be found in any cavity of the heart, and neonatal hemangioma commonly occurs in the RA. However, most CHs are in the ventricle, especially in the lateral wall of the LV (6). A CH rooted in the left atrial appendage is particularly rare, and only several cases have been identified in previous studies (Table 1) (7-14). As a result, our case can be added to this body of literature.

Table 1

Characteristics of cavernous hemangioma of the left atrial appendage in the published literature

Reference Year Author Number of cases Gender Age (years) Clinical presentation Imaging method Morphologic feature Size (cm) Pathological type Therapeutic method Prognosis
(7) 2009 Chiappini 1 Male 65 Syncope and paroxysmal atrial fibrillation TTE Encapsulated, a wild sessile implant basis 5.0×3.0 Cavernous Median sternotomy Without complications
(8) 2015 Darwazah 1 Male 62 Cardiac arrest induced by spinal anesthesia – A wide base with no stalk 1.0×1.0 Cavernous Resection operation –
(9) 2009 Acikel 1 Male 71 Cerebrovascular ischemia TTE Left atrial appendage and right atrium 4.0×1.0 Cavernous Resection operation No recurrence
2.5×1.0
(10) 2022 Fujita 1 Female 76 Cough, dyspnea, and leg oedema Thoracoscopic pericardial fenestration Left atrial appendage 2 Capillary Antero-axillary thoracotomy No pericardial or pleural effusions
(11) 2003 Oshima 1 Male 70 Asymptomatic X-ray Left appendage and protruding into the pericardial cavity 4.5×3.6 Capillary and cavernous Resection operation –
(12) 2021 Suzuki 1 Female 66 Hypertension X-ray Left appendage 5 Cavernous Median sternotomy Without complications
(13) 2013 Takahashi 1 Male 61 Asymptomatic, presenting exertional dyspnea with enlargement of tumor X-ray Left appendage 6.3×5.0 Mainly cavernous type hemangioma with mixed capillary type Median sternotomy Without complications
(14) 2004 Sata 1 Male 72 Recurrent pericardial tamponade TEE Left appendage and growing into the pericardial cavity 4.5×4.5 A combination of cavernous- and venous-type Left anterolateral thoracotomy by way of the fourth intercostal space No pericardial effusion
This case 2022 – 1 Male 60 None CT Left appendage, pedicled 6.5×9.0×7.0 Cavernous Resection operation Without complications

TTE, transthoracic echocardiography; TEE, transesophageal echocardiography; CT, computed tomography.

Patients of all ages can develop CH. Based on the related literature, CH patients are usually approximately 40 years old, with a small male predominance (15). However, our patient was 67 years old and had a left atrial appendage hemangioma. Most affected patients are asymptomatic, but some may show atypical chest pain, abnormal conduction, coronary artery insufficiency, congestive heart failure, pericarditis, pericardial effusion, tamponade, and thromboembolism, among others (3). The patient’s clinical presentations can be vastly different due to the location, size, activity, infiltration, or the speed of growth. Endocardial hemangiomas often present as soft tissue masses, which may contain mucoid components. They often protrude into the heart cavities and lead to hemodynamic changes, thrombosis, obstruction of the outflow tract, valve regurgitation, arrhythmia, or embolism. Furthermore, CHs may also present as Kasabach-Merritt syndrome, which is characterized by multisystemic hemangiomas, thrombocytopenia, and consumptive clotting disorder (3).

Histologic and pathologic features

Histologically, CHs are classified as capillary (numerous capillaries without red blood cells in the lumen), cavernous (thin-walled dilated capillaries with multiple red blood cells in the lumen), or arteriovenous (composed of thick-walled dysplastic arteries, venous-like vessels, and capillaries) (3). Capillary hemangiomas and cavernous hemangiomas are adjacent to the endocardium and have a broad base or thin stalk attached to the wall of the heart but do not infiltrate the myocardium. Moreover, most CHs are located in the myocardium and bear some similarities to the intermuscular hemangiomas of skeletal muscle.

Radiologic features

Echocardiography is the first option for imaging examination because it can be used to demonstrate the location and size of the lesions, and enables a qualitative diagnosis. However, computed tomography (CT) and magnetic resonance imaging (MRI) are superior methods for illustrating the invasion of the lesions. The echocardiographic findings of CHs are hyperechoic masses or nodules. CT often exhibits uneven and low-density lesions with lower density (cystic lesions) and high density (small and patchy calcification) in the tumor. On CECT, CHs display uneven and avid enhancement. Cavernous hemangiomas often show delayed enhancement with gradual filling from the edge to the center, which is similar to cavernous hemangiomas of the liver. On CMR, hemangiomas manifest as an equal signal on T1-weighted imaging (T1WI) and a high signal on T2-weighted imaging (T2WI) due to the slow blood flow. Over the course of echo, the signal increases on T2WI, which is known as the “bulb sign”. The manifestation on contrast-enhanced MRI is similar to that on CECT (16). CMR also provides excellent contrast resolution and multiplanar capability, facilitating qualitative diagnosis and optimal anatomical evaluation of CHs. This anatomical information is especially useful for pre-surgical planning.

Differential diagnosis

Myxomas are the most frequent PCT, accounting for more than 50% of all cases. Papillary fibroelastomas represent the most common valvular tumor. Cardiac rhabdomyomas constitute up to 90% of cardiac tumors in infants and children aged less than 12 months. As a result, a careful differential diagnosis should be performed when providing a preliminary diagnosis of CH.

Myxomas are the most common benign cardiac tumor, accounting for almost half of all PCTs (3,6). Approximately three-quarters of all myxomas arise in the LA, with 23% occurring in the RA, and they often have a narrow pedicle attached to the oval fossa of the atrial septum (3,6). On echocardiography, the lesion appears as a hypoechoic or hyperechoic mobile mass. On CT, the lesion is heterogeneous with areas of high density (calcification) and/or low density (cystic degeneration and necrosis). On CMR, myxomas are typically demonstrated as an equal signal or heterogeneous intensity on T1WI and a high signal or heterogeneous intensity on T2WI. Sometimes, myxomas also show areas of decreased signal, which is attributable to calcification or hemosiderin (3,6). There is an uneven and slight enhancement on gadolinium enhancement.

Papillary fibroelastomas represent the most common valvular tumor (approximately 75% of all cardiac valvular tumors). They are also the second most common benign cardiac tumor (accounting for 16% of all patients) (3,6). A papillary fibroelastoma typically originates from the free edge of the valve, especially in the aortic valve, and is usually medially echoic on echocardiography but is not visible on CT. It presents as isointense on T1WI and T2WI images, with significant enhancement on gadolinium enhancement.

Rhabdomyomas are the most common PCT in children, accounting for 40–60% of cases (3,6). A rhabdomyoma often manifests as multiple masses in the myocardium of the LV or the interventricular septum, and hemorrhage and calcification are rarely seen. On echocardiography, a rhabdomyoma is a hyperechoic mass but is indistinguishable on CT, as the density of the mass is similar to that of the normal myocardium. Rhabdomyomas are isointense or slightly hyperintense on T1WI and hyperintense on T2WI images, and there is no significant enhancement on gadolinium enhancement.

CHs should also be differentiated from primary cardiac sarcomas, which account for most malignant PCTs (about 30%), and are the second most common PCT (about 10%) (3,6). A detailed description of some PCTs is summarized in Table 2.

Table 2

Clinical and imaging features of hemangioma, common benign, or malignant cardiac tumors

Cardiac masses Benign or malignant Percentage (%) Susceptible age Site Morphological variation US CT CMR
Cine-MR imaging T1WI T2WI LGE
Hemangioma Benign 5–10 All ages Anywhere in cardiac or pericardium (especially the ventricle) Polypoid or pedicled, endocardial or intramyocardial Hyperechoic Heterogeneous with calcification or cystic lesions Hyperintense with focal ventricular wall motion abnormality Iso- or slightly hyperintense Hyperintense Heterogeneous prolonged enhancement
Myxoma Benign 50 30–60 years Fossa ovale of atrial septum (left atrium 75%, right atrium 23%) Calcification, cystic degeneration or necrosis Hypoechoic or hyperechoic mobile mass Heterogeneous with calcification Mobile mass Isointense, heterogeneous Hyperintense, heterogeneous Heterogeneous enhancement
Papillary fibroelastoma Benign 16 60–80 years The free edge of the valve, especially the aortic valve Small with narrow stalk, calcification rare Medial echo Usually not visible Hyperintense and movable valve mass with thin stalk Isointense Isointense Significant enhancement
Rhabdomyoma Benign 1 Infants and children Ventricle or the interventricular septum Lobulated nodules and multiple Hyperechoic Isodensity Slightly hyperintense, intramural mass Isointense or slightly hyperintense Hyperintense None
Angiosarcoma Malignant 30 Middle-aged men Almost in the right atrium Circumscribed or diffusely infiltrative, hemorrhage and necrosis Protruding into right atrium, hemorrhagic effusion Heterogeneous Heterogeneous, hemorrhage and necrosis Heterogeneous hyperintense “cauliflower” Heterogeneous hyperintense “cauliflower” Avid enhancement with “Sunray” aspect
Synovial sarcoma Malignant 10–15 Approximately 34.8 years, M:F =3.4:1 Pericardium, right atrium Irregular and lobulated Isoechoic Low density Heterogeneous mass with poly cysts or septa Hypointense or isointense Hyperintense mainly, heterogeneous Heterogeneous enhancement
Rhabdomyosarcoma Malignant 10 Infants, children, M:F =1:1.4 Anywhere in the hear Smooth or irregular contour, central necrosis Multiple, valves and pericardium invasion Low density Isointense Isointense Isointense Heterogeneous enhancement

US, ultrasonography; CT, computed tomography; CMR, cardiac magnetic resonance; T1WI, T1-weighted imaging; T2WI, T2-weighted imaging; LGE, late gadolinium enhancement.

Treatment and prognosis

Although a CH is a benign tumor, surgery should be performed as soon as possible due to the risk of developing angiosarcoma and the possibility that asymptomatic CHs may suddenly cause life-threatening complications. If a cardiac tumor is established, the patient should be treated at an interdisciplinary center. Surgeons, oncologists, radiotherapists, and radiologists need to cooperate comprehensively. On the one hand, surgery is beneficial for diagnosis; on the other hand, it can reduce the risk of rupture or pulmonary embolism (17). Regarding CHs with a clear boundary, radical surgical resection should be performed as far as possible, regardless of tumor size. Patients generally recover well after surgery. A large, extensive CH is potentially at risk of recurrence and often has an unfavorable prognosis because it cannot be completely resected. All patients should be followed up regularly after surgery to determine whether there are new or recurrent lesions.

Conclusions

Hemangioma of the left atrial appendage is rarely encountered clinically. However, comprehensive knowledge of this condition may be helpful in the formulation of a diagnosis and for differential diagnoses.

Acknowledgments

Funding: This study was supported by grants from the Sichuan Provincial Commission of Health (Nos. 18PJ138, 19PJ283, 19PJ284, and 20PJ284), the Sichuan Provincial Department of Science and Technology (No. 2019YFQ0028), the Science and Technology Association of Suining City (Nos. 6 and 10), the Suining Central Hospital (No. 2021y09), and the Science and Technology Bureau of Mianyang City (No. 2020YJKY004).

Footnote

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-22-460/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was provided by the patient for publication of this case report and the accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

References

  1. Burke A, Tavora F. The 2015 WHO Classification of Tumors of the Heart and Pericardium. J Thorac Oncol 2016;11:441-52. [Crossref] [PubMed]
  2. Maleszewski JJ, Basso C, Bois MC, Glass C, Klarich KW, Leduc C, Padera RF, Tavora F. The 2021 WHO Classification of Tumors of the Heart. J Thorac Oncol 2022;17:510-8. [Crossref] [PubMed]
  3. Grebenc ML, Rosado de Christenson ML, Burke AP, Green CE, Galvin JR. Primary cardiac and pericardial neoplasms: radiologic-pathologic correlation. Radiographics 2000;20:1073-103; quiz 1110-1, 1112.
  4. Koçak H, Ozyazicioğlu A, Gündoğdu C, Sevimli S. Cardiac hemangioma complicated with cerebral and coronary embolization. Heart Vessels 2005;20:296-7. [Crossref] [PubMed]
  5. Thilak R, Sivanesan A, Munuswamy H, Toi PC. A Giant Right Atrial Hemangioma- Case Report. Cureus 2022;14:e24622. [Crossref] [PubMed]
  6. Li X, Chen Y, Liu J, Xu L, Li Y, Liu D, Sun Z, Wen Z. Cardiac magnetic resonance imaging of primary cardiac tumors. Quant Imaging Med Surg 2020;10:294-313. [Crossref] [PubMed]
  7. Chiappini B, Gregorini R, Vecchio L, Petrella L, Di Pietrantonio F, Giancola R, Mazzola A. Cardiac hemangioma of the left atrial appendag: a case report and discussion. J Card Surg 2009;24:522-3. [Crossref] [PubMed]
  8. Darwazah AK, Zagha R. Cavernous hemangioma of the left atrial appendage. Asian Cardiovasc Thorac Ann 2015;23:1005-6. [Crossref] [PubMed]
  9. Acikel S, Dogan M, Akdemir R, Kilic H, Yesilay AB, Cagirci G. Multisided cardiac hemangiomas mimicking biatrial thrombus: atypically located cardiac hemangiomas of left atrial appendage and right atrium. J Am Soc Echocardiogr 2009;22:434.e7-9. [Crossref] [PubMed]
  10. Fujita T, Kawaguchi Y, Kataoka Y, Hanaoka J. Cardiac Hemangioma Producing Pericardial Effusion Detected on Thoracoscopic Pericardial Fenestration. Ann Thorac Surg 2022;113:e359-61. [Crossref] [PubMed]
  11. Oshima H, Hara M, Kono T, Shibamoto Y, Mishima A, Akita S. Cardiac hemangioma of the left atrial appendage: CT and MR findings. J Thorac Imaging 2003;18:204-6. [Crossref] [PubMed]
  12. Suzuki T, Uchida H, Fukuhara S, Kanki S, Ozawa H, Daimon M, Katsumata T. Cavernous Hemangioma Originating in the Left Atrial Appendage:Report of a Case. Kyobu Geka 2021;74:237-40. [PubMed]
  13. Takahashi A, Sakurai M, Fujikawa T. Resection of a giant cardiac hemangioma. Gen Thorac Cardiovasc Surg 2013;61:353-5. [Crossref] [PubMed]
  14. Sata N, Moriyama Y, Hamada N, Horinouchi T, Miyahara K. Recurrent pericardial tamponade from atrial hemangioma. Ann Thorac Surg 2004;78:1472-5. [Crossref] [PubMed]
  15. Thomas JE, Eror AT, Kenney M, Caravalho J Jr. Asymptomatic right atrial cavernous hemangioma: a case report and review of the literature. Cardiovasc Pathol 2004;13:341-4. [Crossref] [PubMed]
  16. Domoto S, Kimura F, Uwabe K, Koike H, Tabata M, Iguchi A, Niinami H. Diagnostic features of cardiac cavernous hemangioma in the right ventricle on magnetic resonance imaging. Gen Thorac Cardiovasc Surg 2017;65:40-3. [Crossref] [PubMed]
  17. Cina SJ, Smialek JE, Burke AP, Virmani R, Hutchins GM. Primary cardiac tumors causing sudden death: a review of the literature. Am J Forensic Med Pathol 1996;17:271-81. [Crossref] [PubMed]
Cite this article as: Duan Q, Li H, Yan G, Yang Y, Deng J, Yang L, McClure MA, Bhetuwal A, Yan J, Yang G, Li Y, Zhao L, Fan X. Giant cavernous hemangioma of the left atrial appendage: a case description and literature review. Quant Imaging Med Surg 2023;13(1):538-545. doi: 10.21037/qims-22-460

Article Options

Download Citation

Share