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Pediatric Cardiac Tumors Workup

  • Author: Edwin Rodriguez-Cruz, MD; Chief Editor: P Syamasundar Rao, MD  more...
 
Updated: Jan 04, 2016
 

Laboratory Studies

CBC count with differential should be normal in benign cardiac tumors. In malignant tumors, any kind of blood count abnormality can be present in these patients. For example, anemia is not an uncommon finding. Platelet count may be elevated or low, and WBC count may be high.

Erythrocyte sedimentation rate (ESR) is a nonspecific marker for inflammation that may be normal or elevated; however, the ESR could be low in the presence of congestive heart failure (CHF).

Liver function test findings are occasionally elevated because of severe right-sided heart failure or metastases.

Creatine kinase-myocardial band (CK-MB) fraction levels have been found to be higher in patients who have both ventricular dysfunction and a cardiac tumor but not necessarily primary malignancies from the heart.

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Imaging Studies

Echocardiography

This is the most cost-effective of all tests used to evaluate the anatomy of the heart. Echocardiography can provide important information about size, extension and/or invasion within and outside the heart, valvar involvement and/or competency, ventricular function, and pericardial effusion. Refer to the following images

Echocardiographic parasternal long-axis view showi Echocardiographic parasternal long-axis view showing a large mass within the left ventricular cavity invading or connected to the anterior mitral valve leaflet. AO=Aorta, RV=Right ventricle, LA=Left atrium, Arrow=Left ventricular mass.
Echocardiographic subcostal view of a patient with Echocardiographic subcostal view of a patient with a large mass within the left ventricular cavity invading or connected to the anterior mitral valve leaflet. Mass within the left ventricle fills most of the ventricular cavity. LA=Left atrium, RA=Right atrium, RV=Right ventricle, Arrow=Left ventricular mass.
Echocardiographic parasternal long-axis view demon Echocardiographic parasternal long-axis view demonstrating a rounded mass in the area of the right ventricular outflow tract. Mass was not causing any outflow obstruction. RV=Right ventricle, LV= Left ventricle, AO=Aorta, Arrow=Right ventricular mass.
Echocardiographic apical 5 chamber view of a 1 mon Echocardiographic apical 5 chamber view of a 1 month old patient with several ventricular tumors filling the ventricular cavities in both sides. The patient was having seizures and diagnosed to have tuberous sclerosis. The tumor associated with this disease is rhabdomyoma (see text). RA= Right Atrium; Ao= Aorta; LA= Left Atrium; RV= Right Atrium; LV= Left Ventricle
Follow-up echocardiographic 4 chamber view from th Follow-up echocardiographic 4 chamber view from the same patient as shown in the image above. This echocardiogram was done 8 months after the previous view. Note that the tumors have changed in size and some of them have regressed totally, especially in the right ventricle. The arrows show the remaining areas of tumors seen in both ventricles.

Echocardiography is very sensitive but not specific. Some of these tumors may be too small for echocardiographic detection, especially those leading to dysrhythmias.

Transesophageal echocardiography also is useful in detecting tumors in the left heart, especially in adults.

Chest roentgenography

Cardiomegaly and pulmonary edema may present in cases of obstructive lesions. Certain neoplasms can have calcium deposition detected on the roentgenogram.

Magnetic resonance imaging

Magnetic resonance imaging (MRI) is a powerful tool in diagnosing tumors, evaluating their extent, and seeking metastases.[8] It may delineate the area of the mass better than echocardiography because it has a larger field of observation. Contrast enhancement and multislice imaging can provide a 3-dimensional view of tissue. It also can help differentiate tumors from thrombi.

Computed tomography scanning

Ultrafast computed tomography (CT) scanning is gaining use in this area because it eliminates artifacts of heart motion seen with conventional CT scanning. CT is a powerful tool in evaluating the extent of the tumor and exhibits a high degree of tissue discrimination.

Nuclear medicine imaging

Nuclear medicine studies are used in children to evaluate congenital and developmental disorders of the chest. The same radiopharmaceuticals and imaging techniques are used to evaluate pediatric patients as are used in adults to evaluate cardiac and pulmonary disease, aerodigestive disorders, and pediatric malignancies.[9]

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Other Tests

Electrocardiography

Nonspecific changes are the most common findings on electrocardiography (ECG); however, ST-T segment changes with strain patterns have been noticed in obstructive rhabdomyomas and fibromas. After surgery, these changes return to normal. Other patterns associated with ischemia also may be present. Diffuse low-voltage QRS, especially in the presence of pericardial effusion, can be seen. Arrhythmias may be the first presentation of an intracardiac tumor and occasionally are resistant to medical therapy. Other findings include intraventricular conduction delay and complete bundle branch block.

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Procedures

Endomyocardial biopsy is controversial and seldom useful in children because of the high possibility of false-negative results and the high possibility of metastasis during the procedure if a malignant tumor is present.

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Histologic Findings

Benign tumors

Rhabdomyomas are a glycogenic degeneration of myocardial fibers and are thus considered hamartomas. They contain large vacuolated cells filled with glycogen. Their microscopic hallmark, the spider cell, displays eccentric nuclei, granular cytoplasm with a central cytoplasmic mass, and fine fibrillar processes that radiate to the periphery, giving the appearance of a spider in a web. Because the cytoplasm is rich in glycogen, it stains positive with periodic acid-Schiff reagent. Rhabdomyositis is a rare form of cardiomyopathy. In this pathology, microscopic changes in the muscle and conduction system are evident, yet gross anatomic findings are rare.

Cardiac fibromas consist of elongated fibroblasts, with fibrous tissue and collagen. Occasionally, calcium can be seen using radiography or microscopically within the tumor. Mitosis is rarely seen in this type of tumor.

Myxoma has a characteristic pattern of cells termed lipidic cells embedded in a myxoid stroma rich in glycosaminoglycans. These cells are multinucleated with pink cytoplasm. Typically, they present in clusters surrounding vascular structures. Calcium is present in approximately 10% of patients. Usually, the embolized material has a different pattern from the material at the center of the lesion, and cells resemble embryonic mesenchymal cells, similar to embryonic endocardial cushion tissue.

Teratomas, rarely malignant in children, form from 3 embryonic tissues that usually grow in the anterior mediastinum. These tumors should be differentiated from intrapericardial bronchogenic cysts because they may have the same gross appearance; the histologic difference is that bronchogenic cysts do not contain neuronal tissue, which is usually present in teratomas.

Hemangiomas are classified, based on the predominant type of proliferating vascularity, into hemangiomas (common) or lymphangiomas (extremely uncommon). The tumor contains endothelium-lined spaces that may have blood, lymph, or thrombi.

Malignant tumors

Angiosarcomas have variable vascular channels lined with atypical endothelial cells.

The histologic hallmark of rhabdomyosarcoma is the presence of cross-striations.

Fibrosarcomas are composed of a herringbone pattern of spindle-shaped cells with elongated blunt-ended nuclei and frequent mitoses.

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Contributor Information and Disclosures
Author

Edwin Rodriguez-Cruz, MD Director, Section of Cardiology, Department of Pediatrics, San Jorge Children’s Hospital, Puerto Rico; Private Practice in Interventional Pediatric Cardiology and Internal Medicine, Centro Pedíatrico y Cardiovascular

Edwin Rodriguez-Cruz, MD is a member of the following medical societies: American College of Cardiology, American Heart Association, American Society of Echocardiography, Society for Cardiovascular Angiography and Interventions, Society of Pediatric Echocardiography, American College of Physicians-American Society of Internal Medicine, American Medical Association, Puerto Rico Medical Association

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: St Jude's Medical Co.<br/>Received grant/research funds from NOVARTIS for investigator; Received consulting fee from St. Jude Medical Corp. for speaking and teaching.

Coauthor(s)

Robert D Ross, MD Director of Pediatric Cardiology Fellowship Program, Department of Pediatrics, Division of Pediatric Cardiology, Children's Hospital of Michigan; Professor of Pediatrics, Wayne State University School of Medicine

Robert D Ross, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Ameeta Martin, MD Clinical Associate Professor, Department of Pediatric Cardiology, University of Nebraska College of Medicine

Ameeta Martin, MD is a member of the following medical societies: American College of Cardiology

Disclosure: Nothing to disclose.

Chief Editor

P Syamasundar Rao, MD Professor of Pediatrics and Medicine, Division of Cardiology, Emeritus Chief of Pediatric Cardiology, University of Texas Medical School at Houston and Children's Memorial Hermann Hospital

P Syamasundar Rao, MD is a member of the following medical societies: American Academy of Pediatrics, American Pediatric Society, American College of Cardiology, American Heart Association, Society for Cardiovascular Angiography and Interventions, Society for Pediatric Research

Disclosure: Nothing to disclose.

Additional Contributors

Jeffrey Allen Towbin, MD, MSc FAAP, FACC, FAHA, Professor, Departments of Pediatrics (Cardiology), Cardiovascular Sciences, and Molecular and Human Genetics, Baylor College of Medicine; Chief of Pediatric Cardiology, Foundation Chair in Pediatric Cardiac Research, Texas Children's Hospital

Jeffrey Allen Towbin, MD, MSc is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American College of Cardiology, American College of Sports Medicine, American Heart Association, American Medical Association, American Society of Human Genetics, New York Academy of Sciences, Society for Pediatric Research, Texas Medical Association, Texas Pediatric Society, Cardiac Electrophysiology Society

Disclosure: Nothing to disclose.

Acknowledgements

Rosa M Cintrón-Maldonado, MD Clinical Instructor of Pediatrics, Department of Pediatrics, San Juan Bautista Medical Center

Rosa M Cintrón-Maldonado, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

References
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Echocardiographic parasternal long-axis view showing a large mass within the left ventricular cavity invading or connected to the anterior mitral valve leaflet. AO=Aorta, RV=Right ventricle, LA=Left atrium, Arrow=Left ventricular mass.
Echocardiographic subcostal view of a patient with a large mass within the left ventricular cavity invading or connected to the anterior mitral valve leaflet. Mass within the left ventricle fills most of the ventricular cavity. LA=Left atrium, RA=Right atrium, RV=Right ventricle, Arrow=Left ventricular mass.
Echocardiographic parasternal long-axis view demonstrating a rounded mass in the area of the right ventricular outflow tract. Mass was not causing any outflow obstruction. RV=Right ventricle, LV= Left ventricle, AO=Aorta, Arrow=Right ventricular mass.
Echocardiographic apical 5 chamber view of a 1 month old patient with several ventricular tumors filling the ventricular cavities in both sides. The patient was having seizures and diagnosed to have tuberous sclerosis. The tumor associated with this disease is rhabdomyoma (see text). RA= Right Atrium; Ao= Aorta; LA= Left Atrium; RV= Right Atrium; LV= Left Ventricle
Follow-up echocardiographic 4 chamber view from the same patient as shown in the image above. This echocardiogram was done 8 months after the previous view. Note that the tumors have changed in size and some of them have regressed totally, especially in the right ventricle. The arrows show the remaining areas of tumors seen in both ventricles.
 
 
 
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