Cardiac tumors overview
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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.
- 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.
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