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==Overview==
==Overview==


The '''primary tumors of the heart''' are [[tumor]]s that arise from the normal tissues that make up the [[heart]].  This is in contrast to secondary tumors of the heart, which are typically either [[metastatic cancer|metastatic]] from another part of the body, or infiltrate the heart via direct extension from the surrounding tissues.
The '''primary tumors of the heart''' are [[tumor]]s that arise from the normal tissues that make up the [[heart]].  This is in contrast to secondary [[tumors]] of the heart, which are typically either [[metastatic cancer|metastatic]] from another part of the body, or infiltrate the heart via direct extension from the surrounding tissues.


==Epidemiology and Demographics==
===History and Symptoms===
The most common primary tumor of the heart is the [[myxoma]].  In surgical series, the myxoma makes up as much as 77% of all primary tumors of the heart.<ref name="MolinaEdwards">{{cite journal | author=Molina JE, Edwards JE, Ward HB | title=Primary cardiac tumors: Experience at the University of Minnesota | journal=Thorac Cardiovasc Surg | year=1990 | pages=183-91 | volume=38 Suppl 2| id=PMID 2237900}}</ref>


===Benign Tumors of the Heart===
Cardiac tumors present with a wide variety of symptoms. Many patients remain asymptomatic, and the tumor is an incidental finding. Some patients may present with symptoms of mimicking those of valve abnormalities such as [[mitral stenosis]].  Patients may also present with [[Arrhythmia|rhythm disturbances]],[[endocarditis]] or even [[heart failure]].
About 80% of primary tumors of the heart are benign. Other benign tumors of the heart include [[rhabdomyoma]]s, [[fibroma]]s, [[hemangioma]]s, [[lipoma]]s, [[paraganglioma]]s, myocytic hamartomas, and [[papillary fibroelastoma]]s<ref name="Edwards-1953134">{{cite journal | author=Edwards FH, Hale D, Cohen A, Thompson L, Pezzella AT, Virmani R. | title=Primary cardiac valve tumors | journal=Ann Thorac Surg | year=1991 | volume=52 | issue=5 | pages=1127-31 | id=PMID 1953134}}</ref>.


===Malignant Tumors of the Heart===
===Physical Examination===
About 20% of primary tumors of the heart are malignant in nature.<ref name="MolinaEdwards"/>  Malignant tumors of the heart include [[rhabdomyosarcoma]]s, [[angiosarcoma]]s, myxosarcomas, [[fibrosarcoma]]s, [[leiomyosarcoma]]s, reticulum cell sarcomas, and [[liposarcoma]]s.  The cardiac sarcomas may occur at any age, but are more commonly seen in individuals in their 20s to 40s.  They occur equally in males and females.


===Tumors of the Heart Valves===
A "tumor plop" (a sound related to movement of the tumor), abnormal heart sounds, or [[murmur]] may be heard. These sounds may change when the patient changes position.
A subset of the primary tumors of the heart are tumors that involve the heart valves. Tumors that affect the heart valves are equally distributed among the four heart valves.<ref name="Edwards-1953134"/>  The vast majority of these are papillary [[fibroelastomas]]. Primary tumors of the heart valves predominate  in males. Primary tumors of the heart heart are generally benign. Heart valve tumors often have symptoms related to the valve, including [[stroke]] and [[TIA]], and infrequently [[sudden cardiac death]].
 
===Electrocardiogram===
There are no specific ECG abnormalities associated with heart tumors. The ECG changes vary depending on the location of the tumor and aid in the diagnosis of tumors when used in conjunction with other diagnostic modalities.
 
==Historical Perspective==
As early as 1559, Dr. Realdo Colombo wrote about heart tumors, and the first successful surgery to remove a left [[atrial myxoma]] was performed in 1954 by Dr. Clarence Crafoord. After the discovery of cardiac [[tumors]], new [[chemotherapeutic agents]] and treatments have been developed.
 
==Classification==
Based on their origin, cardiac tumors are classed as either [[primary]] or [[secondary]].
[[Primary cardiac tumors]] are classified as either [[benign]], [[malignant]], or [[intermediate]].
 
==Pathophysiology==
 
Cardiac tumors' exact pathogenesis remains an enigma. Some cardiac tumors are linked to genetic predispositions or are a part of syndromes.
 
*[[Cardiac rhabdomyomas]] are linked to "Tuberous sclerosis," an autosomal dominant disorder characterized by the development of several organ hamartomas.
 
 
 
*[[Cardiac fibromas]] may be related with [[Gorlin syndrome]], an autosomal dominant disorder characterized by congenital anomalies and a predisposition to tumorigenesis.
 
 
 
*[[Cardiac myxomas]] may be related with [[Carney complex]], an autosomal dominant disorder.
 
 
 
*[[Histiocytoid cardiomyopathy]] is occasionally inherited, however the underlying genetic abnormalities are still unclear.
 
 
 
*The majority of undifferentiated cardiac [[sarcomas]] share the same genetic profile as pulmonary artery intimal sarcomas, which are defined by recurrent mutations in the [[MDM2]], [[PDGFRA]], and [[CDKN2A]] genes.
 
*[[Papillary fibroelastomas]] have been reported following cardiac instrumentation and thoracic irradiation.
 
==Causes==
Numerous genetic abnormalities are linked to cardiac tumors however no direct causes have been established as of yet.
Some genetic disorders closely related with a high incidence of cardiac tumors are [[Tuberous Sclerosis]], [[Gorlin Syndrome]], and [[Carneys Triad]].
 
==Differentiating Cardiac Tumors from other Diseases==
Common cardiac tumor symptoms include fever, weight loss, and other constitutional symptoms. Each tumor has unique traits that distinguish it from others.
 
==[[Cardiac tumors epidemiology and demographics|Epidemiology and Demographics]]==
 
==Risk Factors==
There are no recognized cardiac tumor risk factors. However, HIV increases the risk of [[Kaposi sarcoma]] of the heart. Certain disorders, including [[Gorlin syndrome]], [[Tuberous Sclerosis]], [[LAMB]], [[NAME]], and [[Paragangliomas]], are linked to an elevated risk of cardiac tumors.
The presence of other tumors also increases the risk of secondary tumors in heart
=====[[Cardiac tumors risk factors for embolization|Risk Factors for Embolization]]=====
 
==Screening==
The USPSTF does not suggest screening for heart tumors routinely.
[[Transthoracic echocardiography]] (TTE) is the preferred screening tool for cardiac cancers. Valvular lesions are frequently detected by [[Transesophageal echocardiography]] (TEE).
 
==Natural History, Complications and Prognosis==
Cardiac tumors can originate in numerous ways, resulting in a variety of clinical manifestations. Regardless of the type of heart tumor, cardiac symptoms depend mostly on tumor location and size.
Patients with cardiac tumors can experience systemic constitutional symptoms, such as fever, tiredness, and joint issues. Depending on the location of tumors, particular symptoms may develop. The prognosis for benign cardiac tumors is good, but recurrence is possible, whereas the prognosis for malignant tumors is dismal.
 
==Diagnosis==
Multiple cardiovascular imaging techniques could be utilized to outline the anatomy of the cardiac mass and its potential functional consequences.
 
The primary purpose of cardiovascular imaging is to assess the location, extent, and vascularity of the tumor, as well as any hemodynamic consequences and myopericardial invasion.
 
All patients should receive [[two-dimensional transthoracic echocardiography]] (TTE).
A biopsy provides the definitive diagnosis. The technique for a final diagnosis is carried out using [[percutaneous cardiac biopsy]] and [[transvenous cardiac biopsy]], guided by [[echocardiography]], [[mediastinoscopy]], or [[thoracotomy]].
 
[[Cardiac tumors staging|Staging]] | [[Cardiac tumors history and symptoms|History and Symptoms]] | [[Cardiac tumors physical examination|Physical Examination]] | [[Cardiac tumors laboratory tests|Laboratory Findings]] | [[Cardiac tumors electrocardiogram|Electrocardiogram]] | [[Cardiac tumors chest x ray|Chest X Ray]] | [[Cardiac tumors CT|CT]] | [[Cardiac tumors MRI|MRI]] | [[Cardiac tumors echocardiography or ultrasound|Echocardiography]] | [[Cardiac tumors other imaging findings|Other Imaging Findings]] | [[Cardiac tumors other diagnostic studies|Other Diagnostic Studies]]
 
==Treatment==
The vast majority of cardiac tumors are treated surgically. Primary malignant cardiac tumors have a propensity for fast metastatic dissemination.
 
Systemic [[neoadjuvant therapy]] should be vigorously sought in hemodynamically stable patients with localized disease because it permits: (a) faster removal by lowering the size of the tumor, and (b) a reduction in the likelihood of systemic recurrence.
 
In certain instances, surgery may be an option for malignant tumors. Patients with a solitary heart problem and a controlled primary tumor are the best candidates. The tumor must be amenable to excision while preserving sufficient heart function to sustain life.
 
[[Cardiac tumors medical therapy|Medical Therapy]] | [[Cardiac tumors surgery|Surgery]] | [[Cardiac tumors primary prevention|Primary Prevention]] | [[Cardiac tumors secondary prevention|Secondary Prevention]] | [[Cardiac tumors cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Cardiac tumors future or investigational therapies|Future or Investigational Therapies]]
 
==Case Studies==
[[Cardiac tumors case study one|Case #1]] | [[Lymphoma case study|Case #2: Cardiac lymphoma]]


==References==
==References==
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[[Category:Disease]]
[[Category:Cardiology]]
[[Category:Cardiology]]
[[Category:Types of cancer]]
[[Category:Cardiovascular system]]
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Cardiology]]
[[Category:Surgery]]

Latest revision as of 16:00, 7 July 2022

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

The primary tumors of the heart are tumors that arise from the normal tissues that make up the heart. This is in contrast to secondary tumors of the heart, which are typically either metastatic from another part of the body, or infiltrate the heart via direct extension from the surrounding tissues.

History and Symptoms

Cardiac tumors present with a wide variety of symptoms. Many patients remain asymptomatic, and the tumor is an incidental finding. Some patients may present with symptoms of mimicking those of valve abnormalities such as mitral stenosis. Patients may also present with rhythm disturbances,endocarditis or even heart failure.

Physical Examination

A "tumor plop" (a sound related to movement of the tumor), abnormal heart sounds, or murmur may be heard. These sounds may change when the patient changes position.

Electrocardiogram

There are no specific ECG abnormalities associated with heart tumors. The ECG changes vary depending on the location of the tumor and aid in the diagnosis of tumors when used in conjunction with other diagnostic modalities.

Historical Perspective

As early as 1559, Dr. Realdo Colombo wrote about heart tumors, and the first successful surgery to remove a left atrial myxoma was performed in 1954 by Dr. Clarence Crafoord. After the discovery of cardiac tumors, new chemotherapeutic agents and treatments have been developed.

Classification

Based on their origin, cardiac tumors are classed as either primary or secondary. Primary cardiac tumors are classified as either benign, malignant, or intermediate.

Pathophysiology

Cardiac tumors' exact pathogenesis remains an enigma. Some cardiac tumors are linked to genetic predispositions or are a part of syndromes.

  • Cardiac rhabdomyomas are linked to "Tuberous sclerosis," an autosomal dominant disorder characterized by the development of several organ hamartomas.


  • Cardiac fibromas may be related with Gorlin syndrome, an autosomal dominant disorder characterized by congenital anomalies and a predisposition to tumorigenesis.




  • The majority of undifferentiated cardiac sarcomas share the same genetic profile as pulmonary artery intimal sarcomas, which are defined by recurrent mutations in the MDM2, PDGFRA, and CDKN2A genes.

Causes

Numerous genetic abnormalities are linked to cardiac tumors however no direct causes have been established as of yet. Some genetic disorders closely related with a high incidence of cardiac tumors are Tuberous Sclerosis, Gorlin Syndrome, and Carneys Triad.

Differentiating Cardiac Tumors from other Diseases

Common cardiac tumor symptoms include fever, weight loss, and other constitutional symptoms. Each tumor has unique traits that distinguish it from others.

Epidemiology and Demographics

Risk Factors

There are no recognized cardiac tumor risk factors. However, HIV increases the risk of Kaposi sarcoma of the heart. Certain disorders, including Gorlin syndrome, Tuberous Sclerosis, LAMB, NAME, and Paragangliomas, are linked to an elevated risk of cardiac tumors. The presence of other tumors also increases the risk of secondary tumors in heart

Risk Factors for Embolization

Screening

The USPSTF does not suggest screening for heart tumors routinely. Transthoracic echocardiography (TTE) is the preferred screening tool for cardiac cancers. Valvular lesions are frequently detected by Transesophageal echocardiography (TEE).

Natural History, Complications and Prognosis

Cardiac tumors can originate in numerous ways, resulting in a variety of clinical manifestations. Regardless of the type of heart tumor, cardiac symptoms depend mostly on tumor location and size. Patients with cardiac tumors can experience systemic constitutional symptoms, such as fever, tiredness, and joint issues. Depending on the location of tumors, particular symptoms may develop. The prognosis for benign cardiac tumors is good, but recurrence is possible, whereas the prognosis for malignant tumors is dismal.

Diagnosis

Multiple cardiovascular imaging techniques could be utilized to outline the anatomy of the cardiac mass and its potential functional consequences.

The primary purpose of cardiovascular imaging is to assess the location, extent, and vascularity of the tumor, as well as any hemodynamic consequences and myopericardial invasion.

All patients should receive two-dimensional transthoracic echocardiography (TTE). A biopsy provides the definitive diagnosis. The technique for a final diagnosis is carried out using percutaneous cardiac biopsy and transvenous cardiac biopsy, guided by echocardiography, mediastinoscopy, or thoracotomy.

Staging | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | Chest X Ray | CT | MRI | Echocardiography | Other Imaging Findings | Other Diagnostic Studies

Treatment

The vast majority of cardiac tumors are treated surgically. Primary malignant cardiac tumors have a propensity for fast metastatic dissemination.

Systemic neoadjuvant therapy should be vigorously sought in hemodynamically stable patients with localized disease because it permits: (a) faster removal by lowering the size of the tumor, and (b) a reduction in the likelihood of systemic recurrence.

In certain instances, surgery may be an option for malignant tumors. Patients with a solitary heart problem and a controlled primary tumor are the best candidates. The tumor must be amenable to excision while preserving sufficient heart function to sustain life.

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1 | Case #2: Cardiac lymphoma

References

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