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Ann Thorac Surg 2000;69:S358-S368
© 2000 The Society of Thoracic Surgeons
a Pediatric Cardiovascular Surgery, Children’s Hospital, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, Pennsylvania, USA
Address reprint requests to Dr Myers, Pediatric Cardiovascular Surgery, Children’s Hospital at Milton S. Hershey Medical Center, Pennsylvania State University, 500 University Dr, Hershey, PA 17033
e-mail: jlmyers{at}psghs.edu
Presented at the International Nomenclature and Database Conferences for Pediatric Cardiac Surgery, 1998–1999.
Abstract
The extant nomenclature for cardiac tumors is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Cardiac tumors are characterized as primary, tumors which arise directly from tissues of the heart, and secondary, tumors which arise from tissues distant from the heart, with subsequent spread to the otherwise normal tissues of the heart. Tumor types are noted in the hierarchical scheme. A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends.
I. Background
Classically, cardiac tumors are divided into two categories. Primary lesions are derived directly from the tissues of the heart, and secondary lesions have a primary source associated with another organ system, with subsequent metastatic or contiguous spread to the heart. These lesions are not commonly encountered, and much of the literature describing cardiac tumors has been limited to case reports. No formal method of classifying these tumors has been described at length. The aim of this resource is to include those tumors that have been described in the literature and further subdivide them by the characteristics defined by these reports [1–8]. Though primary cardiac tumors are often further subdivided as benign or malignant tumors, this distinction is not always easily made at the time of diagnosis, and this general classification will be avoided. Each of the lesions described will be subdivided to allow complete representation of the tumor, though this may be limited to the level of coding deemed appropriate by the recording surgeons.
II. Analysis: a unified cardiac tumors nomenclature system
Cardiac tumor hierarchy level 1
Cardiac tumor hierarchy level 1 definitions
Cardiac tumor
An abnormal growth of tissue in or on the heart, demonstrating partial or complete lack of structural organization, and no functional coordination with normal cardiac tissue. Commonly, a mass is recognized which is distinct from the normal structural components of the heart.
Cardiac tumor hierarchy level 2
Cardiac tumor hierarchy level 2 definitions
Cardiac tumor, NOS
A cardiac tumor not otherwise specified.
Cardiac tumor, primary
A cardiac tumor that arises directly from tissues of the heart.
Cardiac tumor, secondary
A cardiac tumor that arises from tissues distant from the heart, with subsequent spread to the otherwise normal tissues of the heart.
Cardiac tumor hierarchy level 3
Cardiac tumor hierarchy level 3 definitions
Cardiac tumor, primary, NOS
A primary cardiac tumor not otherwise specified.
Cardiac tumor, primary, myxoma
A tumor of unknown etiology that appears to be a neoplastic process derived from primitive mesenchymal cells. This lesion has the ability to differentiate along endothelial lines.
Cardiac tumor, primary, papillary fibroelastoma
(Synonym: fibroblastoma). A small, usually solitary mass, composed of papillary gelatinous substance sometimes described as resembling sea anemone with small villous fronds.
Cardiac tumor, primary, rhabdomyoma
Lesions composed of large glycogen-rich cells, with scattered bundles of myofibrils resembling fetal hamartoma. These growths are grossly described as firm grayish nodules distinct from surrounding myocardium.
Cardiac tumor, primary, fibroma
Firm, circumscribed lesions that appear encapsulated on gross inspection, but, microscopically are found to have interdigitations of normal myocardium. These lesions are thought to represent true tumors of fibroblastic origin.
Cardiac tumor, primary, lipoma
Well-encapsulated tumors of mature adipose tissue that may be found anywhere in the heart.
Cardiac tumor, primary, lipomatous hypertrophy of the interatrial septum
Nonencapsulated hypertrophy of fat found within the interatrial septum often difficult to distinguish from true cardiac neoplasm. The hypertrophy of primordial fat within the atrial septum does not constitute a true neoplasm. This condition is often associated with arrhythmias or heart chamber obstruction.
Cardiac tumor, primary, pheochromocytoma
Functionally active lesions derived from chromaffin cells of the sympathetic nervous system that produce excess quantities of catecholamines. Grossly these tumors are described as reddish-brown, soft and lobulated.
Cariac tumor, primary, teratoma
Congenital tumor comprised of tissues derived from multipotential embryonic germ cell layers.
Cardiac tumor, primary, hemangioma
Vascular tumor composed of capillary or cavernous vascular channels with classical finding of tumor blush when evaluated by coronary angiography.
Cardiac tumor, primary, mesothelioma of AV node
(Synonyms: Polycystic tumor, Purkinje tumor, or conduction tumor). Small multicystic tumor classically found in close proximity to the AV node extending anywhere from the atrial septum down through the bundle of His. These lesions are associated with conduction disturbances and arrhythmia.
Cardiac tumor, primary, sarcoma
Malignant tumor arising from connective tissue.
Cardiac tumor, primary, cardiac lymphoma
(Synonyms: Extranodal lymphoma, reticulum cell sarcoma, Hodgkin’s lymphoma, lymphosarcoma). Abnormal growth of lymphatic tissue.
Cardiac tumor, secondary, NOS
A secondary cardiac tumor not otherwise specified.
Cardiac tumor, secondary, infradiaphragmatic tumor extension
(Synonym: Subdiaphragmatic extension). Abdominal or pelvic tumor that spreads cephalad through inferior vena cava to the right atrium.
Cardiac tumor, secondary, metastatic extension
Tumor which originally arises from remote organ system and spreads to heart through direct hematogenous or lymphatic spread, or direct extension from contiguous thoracic structures.
Cardiac tumor hierarchy level 4
Cardiac tumor hierarchy level 4 definitions
Cardiac tumor, primary, myxoma, NOS
A myxoma not otherwise specified.
Cardiac tumor, primary, myxoma, solitary
A myxoma that arises as a solitary tumor isolated to a single heart chamber.
Cardiac tumor, primary, myxoma, multiple
Identification of multiple or multicentric myxoma which may involve a single or multiple cardiac chambers.
Cardiac tumor, primary, myxoma, recurrent
A myxoma found at a previous site of myxoma resection.
Cardiac tumor, primary, myxoma, metastatic
Myxoma found at sites distant to previous site of resection.
Cardiac tumor, primary, papillary fibroelastoma, NOS
A papillary fibroelastoma not otherwise specified.
Cardiac tumor, primary, papillary fibroelastoma, valvular
A papillary fibroelastoma that arises from valvular endocardium.
Cardiac tumor, primary, papillary fibroelastoma, ventricular septum
A papillary fibroelastoma that arises from endocardium overlying the ventricular septum.
Cardiac tumor, primary, papillary fibroelastoma, endocardial surface
A papillary fibroelastoma that arises from endocardium not overlying valvular or ventricular septal tissue.
Cardiac tumor, primary, papillary fibroelastoma, papillary muscle
A papillary fibroelastoma that is found to arise from a papillary muscle.
Cardiac tumor, primary, papillary fibroelastoma, chordae tendineae
A papillary fibroelastoma found to arise directly off the chordae tendineae.
Cardiac tumor, primary, rhabdomyoma, NOS
A rhabdomyoma whose site of origin is not otherwise specified.
Cardiac tumor, primary, rhabdomyoma, atrial
A rhabdomyoma that arises from within the atrial wall at a single site.
Cardiac tumor, primary, rhabdomyoma, ventricular
A rhabdomyoma that arises from within the ventricular wall at a single site.
Cardiac tumor, primary, rhabdomyoma, multiple
A rhabdomyoma that arises from multiple locations within the heart.
Cardiac tumor, primary, fibroma, NOS
A fibroma not otherwise specified.
Cardiac tumor, primary, fibroma, atrial
A fibroma found to arise from atrial tissue.
Cardiac tumor, primary, fibroma, ventricular
A fibroma found to arise from ventricular tissue.
Cardiac tumor, primary, lipoma, NOS
A lipoma of the heart not otherwise specified.
Cardiac tumor, primary, lipoma, pericardial
A primary lipoma of the heart found to arise from the pericardial fat.
Cardiac tumor, primary, lipoma, subendocardial
A primary cardiac lipoma found to arise from the subendocardial fat.
Cardiac tumor, primary, lipoma, subepicardial
A primary cardiac lipoma found to arise from the subepicardial fat.
Cardiac tumor, primary, lipoma, interatrial septum
A primary cardiac lipoma found to arise within the wall of atrial septum.
Cardiac tumor, primary, lipoma, intramyocardial
A primary cardiac lipoma found to arise from within the myocardium.
Cardiac tumor, primary, lipoma, multiple
Primary cardiac lipoma found to arise from various sites within the heart.
Cardiac tumor, primary, pheochromocytoma, NOS
A pheochromocytoma of the heart not otherwise specified.
Cardiac tumor, primary, pheochromocytoma, left atrial
A pheochromocytoma found to arise from the left atrium, most commonly from the roof of the left atrium.
Cardiac tumor, primary, pheochromocytoma, interatrial septum
A pheochromocytoma that arises from the interatrial septum.
Cardiac tumor, primary, pheochromocytoma, anterior surface of heart
A pheochromocytoma found to arise along the anterior surface of the heart.
Cardiac tumor, primary, teratoma, NOS
A teratoma of the heart not otherwise specified.
Cardiac tumor, primary, teratoma, benign
A tumor similar in characteristics to the more traditionally described extracardiac teratoma, composed of multiple tissues which are foreign to those in which the lesion arises. These tumors are usually cystic, composed of multiple germinal tissue derivatives, most commonly ectodermal derivatives, and may have a significant amorphous calcium component.
Cardiac tumor, primary, teratoma, malignant
A mediastinal tumor with characteristics of a teratoma, which contains frank histologic evidence of malignancy, or clinically is found to demonstrate evidence of aggressive local invasion.
Cardiac tumor, primary, hemangioma, NOS
A hemangioma of the heart not otherwise specified.
Cardiac tumor, primary, hemangioma, right ventricle
A hemangioma found to arise within the right ventricular wall.
Cardiac tumor, primary, hemangioma, left ventricle
A hemangioma found to arise within the left ventricular wall.
Cardiac tumor, primary, hemangioma, multiple
Hemangioma found to arise from multiple locations within the heart.
Cardiac tumor, primary, sarcoma, NOS
A sarcoma of the heart not otherwise specified.
Cardiac tumor, primary, sarcoma, angiosarcoma
A sarcoma of the heart that microscopically demonstrates disorganized vascular channels lined with anaplastic endothelial cells. These tumors most commonly present grossly as bulky lesions that aggressively invade adjacent local structures.
Cardiac tumor, primary, sarcoma, rhabdomyosarcoma
A sarcoma of the heart that demonstrates a muscle-like appearance on microscopic evaluation, and is associated with bulky intracardiac chamber disease and aggressive locally invasive behavior.
Cardiac tumor, primary, sarcoma, mesothelioma
A sarcoma of the heart that is comprised of stroma with a histologic appearance of mesothelial derived cells.
Cardiac tumor, primary, sarcoma, fibrosarcoma
A sarcoma of the heart that is comprised of stroma with a histologic appearance of fibroblasts mixed with spindle cells.
Cardiac tumor, primary, sarcoma, osteosarcoma
Extraskeletal osteosarcoma composed of a combination of osteoid and fibrous stroma that may contain foci of metaplastic bone.
Cardiac tumor, primary, sarcoma, neurogenic sarcoma
A sarcoma of the heart that is comprised of stroma with a histologic appearance of neurologically derived cells.
Cardiac tumor, primary, sarcoma, leiomyosarcoma
A sarcoma of the heart that is comprised of stroma with a histologic appearance of smooth muscle cells.
Cardiac tumor, primary, sarcoma, liposarcoma
A sarcoma of the heart that is comprised of stroma with a histologic appearance of fat cells.
Cardiac tumor, primary, sarcoma, chondrosarcoma
A sarcoma of the heart that is comprised of stroma with a histologic appearance of chondroblasts.
Cardiac tumor, primary, sarcoma, synovial sarcoma
A sarcoma of the heart that is comprised of stroma with a histologic appearance of synovial cells.
Cardiac tumor, primary, cardiac lymphoma, NOS
A lymphoma of the heart not otherwise specified.
Cardiac tumor, primary, cardiac lymphoma, atrial
Extranodal primary cardiac lymphoma localized to an atrial chamber.
Cardiac tumor, primary, cardiac lymphoma, ventricular
Extranodal primary cardiac lymphoma localized to a ventricular chamber.
Cardiac tumor, primary, cardiac lymphoma, pericardial
Extranodal primary cardiac lymphoma localized to the pericardium.
Cardiac tumor, secondary, infradiaphragmatic tumor extension, NOS
A tumor involving the heart extending from an origin below the diaphragm not otherwise specified.
Cardiac tumor, secondary, infradiaphragmatic tumor extension, renal cell
A renal cell tumor with caval extension from the kidney to the level of the heart.
Cardiac tumor, secondary, infradiaphragmatic tumor extension, hepatic
A primary hepatic tumor that extends through the hepatic veins and inferior vena cava to the level of the heart.
Cardiac tumor, secondary, infradiaphragmatic tumor extension, adrenal
An adrenal tumor with venous extension through the inferior vena cava to the level of the heart.
Cardiac tumor, secondary, infradiaphragmatic tumor extension, leiomyomatosis
A condition consisting of intravenous extension of benign leiomyoma involving the uterus or walls of the large veins, which extend by way of the inferior vena cava to the level of the heart.
Cardiac tumor, secondary, metastatic extension, NOS
Metastatic tumor involving the heart not otherwise specified.
Cardiac tumor, secondary, metastatic extension, leukemia
Discrete nodules consisting of microscopic foci of neoplastic growth associated with systemic manifestations of leukemia.
Cardiac tumor, secondary, metastatic extension, lymphoma
Lymphoma involving the heart that is metastatic from a distant primary lesion.
Cardiac tumor, secondary, metastatic extension, bronchogenic carcinoma
Bronchogenic carcinoma involving the heart that is metastatic from a distant primary lesion.
Cardiac tumor, secondary, metastatic extension, melanoma
Melanoma involving the heart that is metastatic from a distant primary lesion.
Cardiac tumor, secondary, metastatic extension, breast carcinoma
Breast carcinoma involving the heart that is metastatic from a distant primary lesion.
Cardiac tumor, secondary, metastatic extension, sarcoma
Sarcoma involving the heart that is metastatic from a distant primary lesion.
Cardiac tumor hierarchy level 5
Cardiac tumor hierarchy level 5 definitions
Cardiac tumor, primary, myxoma, solitary, NOS
A solitary primary myxoma not otherwise specified.
Cardiac tumor, primary, myxoma, solitary, left atrium
A solitary primary myxoma that is found to arise from the left atrium.
Cardiac tumor, primary, myxoma, solitary, right atrium
A solitary primary myxoma that is found to arise from the right atrium.
Cardiac tumor, primary, myxoma, solitary, left ventricle
A solitary primary myxoma that is found to arise from the left ventricle.
Cardiac tumor, primary, myxoma, solitary, right ventricle
A solitary primary myxoma that is found to arise from the right ventricle.
Cardiac tumor, primary, myxoma, solitary, pulmonary valve
A solitary primary myxoma that is found to arise from the pulmonary valve.
Cardiac tumor, primary, myxoma, solitary, aortic valve
A solitary primary myxoma that is found to arise from the aortic valve.
Cardiac tumor, primary, myxoma, solitary, mitral valve
A solitary primary myxoma that is found to arise from the mitral valve.
Cardiac tumor, primary, myxoma, solitary, tricuspid valve
A solitary primary myxoma that is found to arise from the tricuspid valve.
Cardiac tumor, primary, myxoma, solitary, pulmonary artery
A solitary primary myxoma that is found to arise from the pulmonary artery.
Cardiac tumor, primary, myxoma, solitary, pulmonary vein
A solitary primary, myxoma that is found to arise from a pulmonary vein.
Cardiac tumor, primary, myxoma, solitary, vena cava
A solitary primary myxoma that is found to arise from the vena cava.
Cardiac tumor, primary, papillary fibroelastoma, valvular, NOS
A primary papillary fibroelastoma arising from valvular endothelium not otherwise specified.
Cardiac tumor, primary, papillary fibroelastoma, valvular, aortic valve
A primary papillary fibroelastoma arising from valvular endothelium that arises from the aortic valve.
Cardiac tumor, primary, papillary fibroelastoma, valvular, pulmonary valve
A primary papillary fibroelastoma arising from valvular endothelium that arises from the pulmonary valve.
Cardiac tumor, primary, papillary fibroelastoma, valvular, mitral valve
A primary papillary fibroelastoma arising from valvular endothelium that arises from the mitral valve.
Cardiac tumor, primary, papillary fibroelastoma, valvular, tricuspid valve
A primary papillary fibroelastoma arising from valvular endothelium that arises from the tricuspid valve.
Cardiac tumor, primary, rhabdomyoma, atrial, NOS
An atrial rhabdomyoma not otherwise specified.
Cardiac tumor, primary, rhabdomyoma, atrial, left atrial, sporadic
A rhabdomyoma that arises from the left atrium without the associated manifestations of tuberous sclerosis.
Cardiac tumor, primary, rhabdomyoma, atrial, left atrial, with tuberous sclerosis
A rhabdomyoma that arises from the left atrium in the presence of systemic findings consistent with tuberous sclerosis.
Cardiac tumor, primary, rhabdomyoma, atrial, right atrial, sporadic
A rhabdomyoma that arises from the right atrium without the associated manifestations of tuberous sclerosis.
Cardiac tumor, primary, rhabdomyoma, atrial, right atrial, with tuberous sclerosis
A rhabdomyoma that arises from the right atrium in the presence of systemic findings consistent with tuberous sclerosis.
Cardiac tumor, primary, rhabdomyoma, ventricular, NOS
A ventricular rhabdomyoma not otherwise specified.
Cardiac tumor, primary, rhabdomyoma, ventricular, left ventricular, sporadic
A rhabdomyoma that arises from the left ventricle without the associated manifestations of tuberous sclerosis.
Cardiac tumor, primary, rhabdomyoma, ventricular, left ventricular, with tuberous sclerosis
A rhabdomyoma that arises from the left ventricle in the presence of systemic findings consistent with tuberous sclerosis.
Cardiac tumor, primary, rhabdomyoma, ventricular, right ventricular, sporadic
A rhabdomyoma that arises from the right ventricle without the associated manifestations of tuberous sclerosis.
Cardiac tumor, primary, rhabdomyoma, ventricular, right ventricular, with tuberous sclerosis
A rhabdomyoma that arises from the right ventricle in the presence of systemic findings consistent with tuberous sclerosis.
Cardiac tumor, primary, rhabdomyoma, multiple, NOS
Primary cardiac rhabdomyoma that are found to arise from multiple cardiac chambers that are not otherwise specified.
Cardiac tumor, primary, rhabdomyoma, multiple, sporadic
Primary cardiac rhabdomyoma that are found to arise from multiple cardiac chambers without the associated manifestations of tuberous sclerosis.
Cardiac tumor, primary, rhabdomyoma, multiple, with tuberous sclerosis
Primary cardiac rhabdomyoma that are found to arise from multiple cardiac chambers in the presence of systemic findings consistent with tuberous sclerosis.
Cardiac tumor, primary, fibroma, atrial, NOS
A primary cardiac fibroma arising within the atrium not otherwise specified.
Cardiac tumor, primary, fibroma, atrial, right atrial wall
A primary cardiac fibroma found to arise from the right atrial wall.
Cardiac tumor, primary, fibroma, atrial, left atrial wall
A primary cardiac fibroma found to arise from the left atrial wall.
Cardiac tumor, primary, fibroma, ventricular, NOS
A primary cardiac fibroma arising within the ventricle not otherwise specified.
Cardiac tumor, primary, fibroma, ventricular, left ventricular free wall
A primary cardiac fibroma found to arise from the left ventricular free wall.
Cardiac tumor, primary, fibroma, ventricular, ventricular septum
A primary cardiac fibroma found to arise from the ventricular septum.
Cardiac tumor, primary, fibroma, ventricular, right ventricular free wall
A primary cardiac fibroma found to arise from the right ventricular free wall.
Cardiac tumor, primary, teratoma, benign, NOS
A tumor demonstrating characteristics of a benign teratoma not otherwise specified.
Cardiac tumor, primary, teratoma, benign, intracardiac
A tumor demonstrating characteristics of a benign teratoma that arises from within the heart.
Cardiac tumor, primary, teratoma, benign, intrapericardial
A tumor demonstrating characteristics of a benign teratoma that arises from within the pericardium.
Cardiac tumor, primary, teratoma, malignant, NOS
A tumor demonstrating characteristics of a malignant teratoma not otherwise specified.
Cardiac tumor, primary, teratoma, malignant, intracardiac
A tumor demonstrating characteristics of a malignant teratoma that arises from within the heart.
Cardiac tumor, primary, teratoma, malignant, intrapericardial
A tumor demonstrating characteristics of a malignant teratoma that arises from within the pericardium.
Cardiac tumor, primary, sarcoma, angiosarcoma, NOS
A primary cardiac angiosarcoma not otherwise specified.
Cardiac tumor, primary, sarcoma, angiosarcoma, tricuspid valve
A primary cardiac angiosarcoma that is found to arise from the tricuspid valve.
Cardiac tumor, primary, sarcoma, angiosarcoma, pulmonary valve
A primary cardiac angiosarcoma that is found to arise from the pulmonary valve.
Cardiac tumor, primary, sarcoma, angiosarcoma, mitral valve
A primary cardiac angiosarcoma that is found to arise from the mitral valve.
Cardiac tumor, primary, sarcoma, rhabdomyosarcoma, NOS
A primary cardiac rhabdomyosarcoma not otherwise specified.
Cardiac tumor, primary, sarcoma, rhabdomyosarcoma, mitral obstruction
A primary cardiac rhabdomyosarcoma that presents with mitral valve obstruction.
Cardiac tumor, primary, sarcoma, rhabdomyosarcoma, pulmonic obstruction
A primary cardiac rhabdomyosarcoma that presents with pulmonary outflow tract and/or pulmonary valve obstruction.
Cardiac tumor, primary, sarcoma, fibrosarcoma, NOS
A primary cardiac fibrosarcoma not otherwise specified.
Cardiac tumor, primary, sarcoma, fibrosarcoma, left atrium
A primary cardiac fibrosarcoma that arises from the left atrium.
Cardiac tumor, primary, sarcoma, fibrosarcoma, right atrium
A primary cardiac fibrosarcoma that arises from the right atrium.
Cardiac tumor, primary, sarcoma, fibrosarcoma, left ventricle
A primary cardiac fibrosarcoma that arises from the left ventricle.
Cardiac tumor, primary, sarcoma, fibrosarcoma, right ventricle
A primary cardiac fibrosarcoma that arises from the right ventricle.
Cardiac tumor, primary, sarcoma, fibrosarcoma, multiple
Primary cardiac fibrosarcoma that is found to involve multiple cardiac chambers at the time of presentation.
III. Nomenclature for cardiac tumor treatment options
Cardiac tumor treatment hierarchy level 1
Cardiac tumor treatment hierarchy level 2
Cardiac tumor treatment hierarchy level 3
IV. Diagnosis and procedure short lists
References
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