B P11 C98 Tumors Affecting the Cardiovascular System Flashcards
The most important consideration in confirming the presence of a cardiac tumor is a _____ in a logical manner to establish a clinically reasonable plan of action
(1) High index of suspicion
(2) The integration of symptoms, physical findings, and imaging characteristics
The initial diagnostic test for a patient with concerning symptoms often involves an imaging test, such as _____.
2DED or cMRI
Depending on the characteristics of this mass and the known comorbidities of the patient, additional imaging may be undertaken. These including three-dimensional (3D) echo with or without contrast, cMRI with gadolinium, coronary angiography (to define the presence of coronary artery disease), positron emission testing (PET) to provide staging for cancer, or computed tomography (CT), including a CT angiogram (CTA) to clarify intrathoracic structures.
A differential diagnosis of a cardiac mass is broad and includes _____.
Tumors
Thrombi
Infection
Artifacts
The most important characteristic in the evaluation of a cardiac mass would be evidence of _____ as an indicator of a benign or malignant tumor
Perfusion into the mass
When considering typically encountered clinical scenarios, a patient with new-onset heart failure and severe left ventricular (LV) dysfunction, who has a 2D-echo image that shows an apical mass, a cardiac tumor is quite unlikely.
This suspicion would be firmly established if there was a severe wall motion abnormality in that region, the mass appeared distinct from the myocardial wall and was lobulated. An LV mass with these characteristics is much more likely to be a _____ as opposed to a tumor.
Thrombus
Another scenario involves a patient with a history of melanoma that is metastatic to other organs, who has routine cardiac imaging and a solid mass is seen in an unusual location. Since there is no wall motion abnormality and no significant valvular disease or clinical signs suggestive of infective endocarditis, a mobile mass on the tricuspid valve is very likely to be a ______ to the heart
Metastatic lesion
Another imaging characteristic that provides insight indicating a tumor is present is the behavior of the mass during cardiac motion. If a tumor is infiltrating the myocardium, it is _____ in a normal fashion.
Unlikely to contract
An LV myocardial apical mass contracting similarly to the surrounding tissue is likely to be either _____ as opposed to a cardiac tumor.
Focal hypertrophy or LV noncompaction
If a cardiac mass changes in size during serial imaging, suspicion of a _____ is much higher
Cardiac tumor
Range of clinical findings that may indicate a Cardiac Tumor
- Completely asymptomatic but an incidental abnormality on imaging
- Low grade fevers
- TIA or CVA
- Positional dyspnea
- Weight loss
- Peripheral embolic events
- Chest discomfort
- CHF
- Upper extremity or neck swelling
- Lower extremity venous thrombosis
- Palpitations
- Arrhythmias
- Pericardial effusion/tamponade
The exact nature and location of a mass is critical in the determination of the likelihood that it is a tumor. A classic example of this principle is _____.
Lipomatous hypertrophy of the intraatrial septum
Differential diagnoses of cardiac masses
- Intracardiac thrombus
- Focal myocardial hypertrophy
- LVNC
- Primary cardiac tumor
- Secondary cardiac tumor (Metastasis)
- Lipomatous hypertrophy of the septum
- Cyst
- Imaging artifact
In terms of primary tumors, approximately ___% are benign and these can be grouped as simple or complex, considering the treatment that is typically required.
The approximately ___% of primary cardiac tumors remaining are malignant and usually are pathologically described as sarcomas
80% Benign
20% Malignant (Sarcomas)
_____ cardiac tumors are 30 times more common than a primary neoplasm with an autopsy incidence of 1.7% to 14%18 or 1:100.14
Secondary or metastatic
The majority (>_____%) of the primary cardiac tumors are nonmalignant; however, because of their location these frequently require surgical treatment.
> 80%
______ constitutes about 50% of all benign cardiac tumors in adults, but only a small percentage of such tumors in children.
Myxoma
_____ are the most common benign tumor in children and account for 40% to 60% of the pediatric cases
Rhabdomyomas
Most myxomas (>80%) are most commonly found in the _____ and in decreasing frequencies in the _____.
LA
LA -> RA -> RV -> LV
The incidence of cardiac myxoma peaks at 40 to 60 years of age, with a female to male ratio of approximately 3:1.
Carney’s complex, an autosomal-dominant condition associated with _____.
- Cardiac myxomas
- Myxomas in other regions (cutaneous or mammary)
- Hyperpigmented skin lesions
- Hyperactivity of the adrenal or testicular glands,
- Pituitary tumors
Carney’s complex occurs at a younger age and should be considered when cardiac myxomas are discovered in atypical locations in the heart
The exact origin of myxoma cells remains uncertain, but they are thought to arise from remnants of _____ in the region of the fossa ovalis, which can differentiate along a variety of cell lines.
Subendocardial cells or Multipotential mesenchymal cells
Myxomas typically form a pedunculated mass with a _____ base (85% of myxomas), but sessile forms can also occur.
Short broad base
Classically, myxomas appear _____. The tumor size can range from 1 cm to more than 10 cm, and the surface is smooth in the majority of the cases.
Yellowish, appear white or brownish, and are frequently friable
A _____ form of myxoma has been reported and contains a surface that consists of multiple fine or very fine villous, gelatinous, and fragile extensions that have a tendency to fragment spontaneously and are associated with embolic phenomena
Villous or papillary myxoma
Histologically, myxomas are composed of _____ that may also contain endothelial cells, smooth muscle cells, and other elements surounded within an acid mucopolysaccharide substance.
Spindle and stellate shaped cells with myxoid stroma
When symptoms are present, dyspnea, especially dyspnea that is worse while lying on the ____, should alert the astute clinician to the possibility of a myxoma.
Left side
Most clinical presentations related to myxoma result from ______.
MV obstruction (syncope, dyspnea, and pulmonary edema)
Followed by:
Embolic manifestations
A _____ may potentially be heard (a low-pitched diastolic sound heard as the tumor prolapses into the left ventricle)
Tumor plop
In one study, a cardiac auscultation abnormality was detected in 64% of patients, and the most common auscultation findings are a _____.
Systolic murmur (50%)
Loud first heart sound (32%)
Opening snap (26%)
Diastolic murmur (15%)
The reason for the systolic murmur may be caused by damage to the valves, failure of the leaflets to coapt, or narrowing of the outflow tract by the tumor
A diastolic murmur is present due to obstruction of the mitral valve from the tumor.
Tumor plop may be confused with a mitral opening snap or a third heart sound and can be detected in up to 15% of cases.
Laboratory test abnormalities in myxomas may include ______, which is present in approximately 75% of the patients.
Anemia
Elevated serum gamma globulin
Elevated ESR
Elevated serum CRP
A 2D echo usually should demonstrate a mass in the atrium, with the stalk attached to the _____ but myxomas have been reported in all chambers of the heart
Interatrial septum
The only definitive treatment of cardiac myxoma is _____.
Surgical removal
Generally, the myxoma is surgically excised using cardiopulmonary bypass and cardioplegic arrest
The recurrence rate of myxoma has varied but one large experience suggests that is quite low and may be below 1%
Valvular structures may have a papillary fibroelastoma attached, which is often found incidentally.
These are small in size, typically less than 2 cm, and most commonly occur on the _____.
Aortic valve followed by the mitral valve
Fibroelastomas may result in embolic phenomena, and when situated on the aortic valve or the left ventricle, can cause _____.
Coronary ostial occlusion
Grossly, fibroelastomas have a characteristic _____, and histologically the tumor has an _____.
Gross:
Frond like appearance, resembling a sea anemone
Histologically:
Inner central core of collagen surrounded by a layer of acid mucopolysaccharides and covered by endothelial cells
For the most part, _____ is recommended for left-sided papillary fibroelastoma primarily because of the high likelihood of systemic embolism, can lead to stroke, myocardial infarction, peripheral embolism, and even sudden death
Complete surgical resection
The decision for surgery in right-sided disease is more difficult as the number of asymptomatic right heart fibroelastomas is unknown and recommendation for surgery depends on the exact location, size, and potential risk to the patient
On imaging of fibroelastomas, especially echocardiographic imaging, there is a characteristic ______ that enables it to be differentiated from a vegetation or thrombi
Small, mobile, pedunculated, and very echo dense core
Most often, surgery can be avoided in rhabdomyomas, although if _____ become a symptomatic problem, antiarrhythmics and ultimately surgery may have to be considered.
Arrhythmias
Frequently, these patients are asymptomatic although some patients with rhabdomyoma may present clinically with arrhythmias and heart failure
_____ are usually found in the ventricle and are the most common benign cardiac tumor found in children.
Rhabdomyomas
The majority of these patients have signs of or a family history of tuberous sclerosis.
It is possible these tumors may regress with age but can sometimes grow or appear during puberty.
A lipoma is a rare benign cardiac tumor comprising only 3% of all benign tumors.
These tend to occur in the _____ but may be found anywhere in the heart as well as the pericardium.
LV or RA
Obstruction and compression of the _____ may occur in patients with lipomas involving the right atrium.
SVC
Although frequently asymptomatic, lipomas may grow large enough to cause _____ symptoms and require surgical intervention
Obstructive symptoms
Because of their location near the atrioventricular node, these cystic tumors can present with varying degree of heart block or even sudden death.
Cystic tumor of the AV node (“Mesothelioma)
Cardiac MRI is particularly useful in the diagnosis of this tumor
______ are characteristically vascular and maybe endocardial or epicardial
Hemangiomas
______ are chromaffin-producing tumors arising from the neural crest cells of the sympathetic and parasympathetic chains.
Only 1% to 2% occur in the chest and most of these are in the posterior mediastinum
Cardiac paragangliomas
Cardiac paragangliomas may be located in the pericardial space with no intracardiac extension but are often located around the _____ involving the cardiac structures and can occur anywhere in the heart.
Roof of the left atrium and aortic root
When paraganglioma is suspected, a coronary CT angiogram or cardiac catheterization is necessary looking for _____ .If present, this is almost pathognomonic for paraganglioma and diagnostic biopsy should be avoided due to the high risk of bleeding
Large feeding vessels
A coronary angiogram in patients with cardiac paraganglioma shows a characteristic _____.
Tumor blush
These tumors are histologically composed primarily of fibroblasts or collagen. Typically these occur in childhood, although it can also occur in adults.
Fibromas
Most often a fibroma is located in the ______, and patients may present with chest pain, pericardial effusion, heart failure, arrhythmias, and sudden death.
Cardiomegaly is frequently seen on chest x-ray, which may also show the ______ within the tumor mass.
Ventricle and IVS
Calcification
A distinguishing feature of fibromas, contrasted to rhabdomyoma, is that there is classically _____ within the tumor.
Calcification
In their select series there was _____% 1-year survival among patients with benign tumors and _____% survival in patients with malignant tumors (primarily sarcoma).
Benign: 100%
Malignant: 50%
Primary cardiac tumors, both benign and malignant, are rare with a meta- analysis covering 22 studies showing an autopsy incidence of approximately 0.02% while primary cardaic _____ make up over 75% of these malignant tumors
Sarcomas
The age of presentation for cardiac sarcomas ranges from 1 to 76 years, with a mean age around 40 years
Angiosarcomas and unclassified sarcomas account for approximately 76% of all cardiac sarcomas, of which ______ are the most common
Angiosarcomas
______ is the most common form of cardiac sarcoma in children.
Rhabdomyosarcoma
Angiosarcomas are predominantly found on the _____ side while osteosarcomas and unclassified sarcomas are predominantly found on the _____ side of the heart.
Angiosarcomas: Right side
Others: Left side
It is generally agreed that _____ is the mainstay of therapy of angiosarcomas. If surgery cannot be done, the1-year survival is less than10%
Complete surgical resection
Additionally, the role of neoadjuvant and adjuvant chemotherapy and radiation therapy are poorly defined as most studies have been small, although recent evidence seems to be favoring both.
Cardiac tumors (sarcomas) commonly cause symptoms by three separate mechanisms:
Obstruction
Embolization
Arrhythmias
Rarely pericardial invasion and tamponade may be the first manifestation of the disease
A clinically practical way to consider primary cardiac sarcoma is by tumor location since this often determines the clinical presentation, urgency of treatment, and surgical options:
(1) Right heart
(2) Left heart
(3) Pulmonary artery
The most common presenting symptoms of cardiac sarcomas include ______ ,followed by chest pain, cough, syncope, hemoptysis, sudden death, fever, embolic events, and cardiac arrhythmias.
Dyspnea
Unfortunately, about 29% of cardiac sarcomas have metastatic disease at the time of presentation, typically in the _____.
Lung
A finding of a cardiac mass with ______ should raise the suspicion of a malignant cardiac tumor.
Pericardial effusion
Regardless of the imaging modalities used, malignant tumors of the heart often involve _____ including the pericardium, epicardium, endocardium, and valve planes.
Invasion of the tumor across tissue boundaries or planes
This feature often distinguishes these tumors from nonmalignant and other normal structures
ECG changes in malignant cardiac tumors are usually ______; however, heart block, ventricular hypertrophy, bundle branch blocks, atrial flutter, and atrial tachycardia may be present in some cases.
Nonspecific
Cross sectional imaging methods, such as CT and MRI, have an important role in the evaluation and further assessment of malignant cardiac tumors, especially in evaluation of _____.
- Myocardial invasion
- Involvement of mediastinal structures
- Tissue characterization
- Vascularity
A _____ is the optimal goal for surgical treatment of malignant cardiac tumors. Once surgical treatment is completed, adjuvant chemotherapy seems prudent although not widely studied but has shown to improve survival over surgery alone in a SEER database analysis.
Complete resection
The most common chemotherapeutic regimen used for cardiac sarcomas is combined ______.
A combination of ______ also showed some response in various sarcomas and can be used as an alternative chemotherapeutic regimen
Doxorubicin + Ifosfamide
Alternative:
* Gemcitabine + Docetaxel
* Ifosfamide + Epirubicin
* Cyclophosphamide + Vincristine + Doxorubicin + Dacarbazine
The presence of _____ is associated with a poor prognosis as is the presence of a _____-sided cardiac sarcoma.
Tumor necrosis and metastases
Right sided sarcoma
Markers of better survival in cardiac sarcomas:
Left sided sarcoma
Complete resection
Negative surgical margins
Low histologic grade
Cardiac metastases can occur either by:
(1) Direct extension
(2) Blood stream
(3) Lymphatics
(4) Intracavitary diffusion through the inferior vena cava (IVC)
______ metastasis (69%) is the most common location followed by epicardial (34%), myocardial (32%), and endocardial metastases (5%).
Pericardial metastasis
The pericardium is most often involved due to direct invasion by the thoracic cancers, including breast and lung cancer
A recent review suggests that _____ is the most common cause of cardiac metastasis followed by esophageal cancer and hematologic malignancy
Lung cancer
Followed by:
Esophageal cancer
Hematologic malignancy
Abdominal and pelvic tumors may reach the right atrium through the IVC.
The most common tumor exhibiting this tendency is _____.
Renal cell carcinoma
A _____ in a cancer patient should raise the suspicion of cardiac metastases
New heart murmur
or
Any new ECG finding without clear symptoms
Typical ECG findings encountered patients with cardiac metastases are:
- ST-T wave changes (mimicking myocardial ischemia or injury)
- New atrial fibrillation or flutter
- Low voltages with electrical alternans indicating a significant pericardial effusion
Treatment of metastatic cardiac tumors is usually _____, as overall prognosis is poor, with >50% of patients dying within one year. Palliative radiotherapy and chemotherapy in chemo-sensitive tumors are recommended.
Palliative
The ECG findings of myocardial injury may indicate an invasion of the ______ by tumor.
Coronary vessels
The differential diagnosis of a pericardial effusion in a patient with a known malignancy includes:
- Malignant effusion
- Radiation-induced or drug-induced pericarditis
- Idiopathic pericarditis
- Infectious (including tuberculosis, fungal, or bacterial)
- Iatrogenic, secondary to procedures
It is estimated that approximately 40% of patients with cancer and a pericardial effusion were found to have either radiation-induced or idiopathic and only a minority actually have malignant effusion
Drug-induced pericarditis is typically seen after high-dose ____ therapy
Anthracycline or cyclophosphamide
SVC obstruction most commonly results from _____.
Tumor infiltration of the vessel wall
or
Thrombotic occlusion
_____ is a late complication of chest irradiation that may be becoming more common because of the longer survival of patients with breast cancer and Hodgkin disease who typically receive chest irradiation.
Constrictive pericarditis
or
Effusive-constrictive pericarditis
Over a period of time, vascular causes have declined and now the most common cause of SVC syndrome is malignancy of which _______ is the most common followed by lymphoma and metastatic cancer
Lung carcinoma
Followed by:
Lymphoma
Metastatic cancer
Malignancy accounts for >85% of causes of SVC syndrome
Other causes of SVC syndrome, which are somewhat benign, account for 3% to 15% of the cases and includes: nonmalignant causes like thrombosis due to use of intravascular devices such as catheters or pacemaker, infection, thymoma, substernal thyroid goiter,and aortic aneurysm.
Other possibilities include disease causing systemic vasculitis (e.g., Behçet disease and radiation- induced fibrosis)
A usual patient presentation of SVCs involves _____.
Facial edema
Arm swelling
Dyspnea
Cough
Facial and neck
edema is most frequently seen, which is worse in the morning and gets better during the day as the patient ambulates.
The blood flow in the venous system is under low pressure and the vessel itself is thin-walled.
Any _____ can cause the SVC to be compressed resulting in reduced blood flow and eventually complete occlusion
- Inflammatory process in the mediastinum
- Enlargement of the lymph nodes or ascending aorta enlargement
Manifestations of supra-azygos SVC obstruction
- Distended arm and neck veins
- Edema of the neck, face and arms
- Congested mucous membranes (mouth)
- Dilated, tortuous vessels on upper part of the chest and back
Manifestations of infra-azygos SVC obstruction
- More severe symptoms but all the features for obstruction distal to entrance of SVC
- Dilatation of collateral vessels on anterior and posterior abdominal wall with downward blood flow into the IVC, then back to the heart
The management of SVC syndrome associated with malignant disease involves _____.
(1) Treatment of the underlying malignancy
(2) Alleviating the obstructive symptoms
The median life expectancy is 6 months, with a range of 1.5 to 9.5 months.
Treatment is guided by the severity of the symptoms and the underlying malignant disease.
In cases of known malignancy, systemic chemotherapy and radiation therapy are typically carried out
If the main cause of SVC obstruction is thrombosis, _____ deployment is an attractive option.
_____ of an obstructed SVC is another option, especially if insufficient diagnostic tissue can be obtained by other measures.
Palliative endovascular stent
Surgical bypass
