dr shreetal rajan nair senior resident, department of cardiology, calicut medical college
TRANSCRIPT
Cardiac tumorsDr Shreetal Rajan Nair
Senior Resident, Department of Cardiology,Calicut Medical College
History 1559 : Columbus , a noted pathologist – first to report 1934 : first report of a primary sarcoma of the heart
diagnosed in a living patient by Barnes1942 : surgical resection of intrapericardial teratoma
by Beck 1951 : The first successful excision of a cardiac tumor
performed by Maurer 1952 : Goldberg diegnosed intracavitary myxoma by
angiocardiography1968 : Shattenberg used echo to diagnose cardiac
tumors
Classification Primary benign (75%)
malignant (25%)
Myxoma – 50%Rhabdomyoma – 20%Fibroma Papillary fibroelastomaLipoma Angiosarcoma RhabdomyosarcomaLeiomyosarcoma Lymphomas
secondary Breast
lungs
Incidence All age groups - 0.002% to 0.03%Pediatric - 0.027% to 0.08%Adults - upto 0.02%
By comparison, metastatic involvement is 20 times more common
Incidence of benign tumors
Shapiro LM. Cardiac tumours: diagnosis and management. Heart 2001; 85: 218 - 22.
Tumors of the heart - 1. Constitutional or systemic
2. Embolic 3. Cardiac 4. Phenomena secondary to metastatic
diseases.
Specific signs and symptoms generally are determined by the location of the tumor, size, friability , mobility in the heart and depends least on the histopathology
Tumors of the heart- clinical presentations
Primary tumors most commonly involve the endocardium, followed by myocardium and then epicardium
Metastatic involvement of heart is reverse that of primary tumors
Endocardium- obstruction to flow of blood through heart
Myocardium - rhythm abnormalities
Epicardium – pericardial effusion, cardiac tamponade
Approach to a cardiac tumorDifferential diagnosis
PUOIE/CONNECTIVE
TISSUE DISEASESVALVULAR LESIONSCCF
CONDUCTION ABNORMALITIES
EMBOLISMSYNCOPEPERICARDIAL
EFFUSION
Diagnostic evaluation
Confirm the diagnosis
locate the lesion within the heart
Whether benign or malignant
Echocardiography Simple, noninvasive technique for initial
evaluation.
Images myocardium and the cardiac chambers Identify the presence of a mass and its mobility.
Information about any obstruction to the circulation & whether the tumor could be a source of emboli
Echocardiography - TEE vs TTE Transthoracic echocardiography – the initial
diagnostic tool
Transesophageal echocardiography (TEE)- more informative.
This is due to the proximity of the esophagus to the heart, the lack of intervening lung and bone, and the ability to use high-frequency imaging transducers that afford superior spatial resolution
CARDIAC MRI VS CTBoth provide noninvasive, high resolution
images of the heart, but MRI generally is preferred.
cMRI -detailed anatomic images T1- and T2-weighted sequences help to
identify the chemical microenvironment within a tumor
CT -useful when calcification is present and MRI is not available or is contraindicated.
CORONARY ANGIOGRAPHY
Mapping the blood supply of tumors arising from the epicardial surfaces
vital to the success of excising such tumors.
Significant involvement of coronary arteries - resection and grafting of such arteries
Role of Transvenous biopsy
Limited data are available on the risks and benefits
considered reasonable if potential benefits outweigh risks and there is diagnostic dilemma
Benign tumorsAround 75 percent of primary cardiac
tumors are benign
In adults, most common- myxomas (50%)
Other common benign lesions- papillary fibroelastomas and
lipomas.
In children, the most common-rhabdomyomas and fibromas
Myxomas Most common primary cardiac neoplasm.
Histology- scattered cells within a mucopolysaccharide stroma which originate from multipotent mesenchymal cells capable of neural and endothelial differentiation .Hallmark histological feature is myxoma (lepidic) cell
Produce VEGF-induction of angiogenesis and the early stages of tumor growth
& IL 6 -responsible for the constitutional features.
Myxomas Typically pedunculated and gelatinous in
consistency; smooth, villous, or friable.
Calcification , necrosis and cystic changes seen
Mostly (90%) solitary
About 35 % of are friable or villous, these tend to present with emboli.
Larger tumors -smooth surface and
associated with cardiovascular symptoms.
Myxomas The cardiovascular manifestations depend
upon the anatomic location of the tumor.
Mostly seen in females 40-60 years of age
80% -originate in the left atrium, most of the remainder is found in the right atrium
constitutional symptoms (eg, weight loss, fever) and laboratory abnormalities
Syndrome Myxoma Characteristics
Syndromic associations
Younger than 40 years
Biatrial ,ventricular , valves
all first degree relatives should be screened
Recurrent – 30%
No gender predilection
LAMB (lentigines, atrial myxomas,mucocutaneous myxomas, and blue nevi),
NAME (nevi, atrial myxomas, myxoid neurofibroma, and ephelides
Carney syndrome (atrial, cutaneous and mammary myxomas, lentigines, blue nevi, endocrine disorders and testicular tumours)
Myxoma Carney syndrome Histological features
Mutations in :
PRKAR1A (protein kinase A regulatory subunit 1A )
MYH8 - myosin isoform
PAS
Vimentin
S100 and NSE - POSITIVITY
Treatment
Prompt resection-due to the risk of embolization or cardiovascular complications, including sudden death
Result of surgical resection is good ( <5% mortality)
Cardiac autotransplantation
Papillary fibroelastomas
Second most common primary cardiac tumor in adults
Their appearance is compared to pom-pom or sea anemones
Clinical featuresSymptoms -caused by embolization of the
tumor/thrombus. most common clinical presentation- stroke
/TIA.
Commonly attached to the aortic valve
30%- incidentally diagnosed by echocardiography, at cardiac surgery, or at autopsy
TREATMENT
SURGERY –
1. patients who have had embolic events or complications directly related to tumor mobility (eg, coronary ostial occlusion)2.Those with highly mobile or large (≥1 cm) tumors
Recurrence not reported
Lipomas
Lipomas &fibrolipomas - characterized by a predominance of benign
fatty cells.
About half of these tumors occur in the subendocardial region.Others in the myocardial and subepicardial regions
They may also occur on valves
Lipomas Symptoms- generally related to local tissue
encroachment (arrhythmias, conduction block, sudden death)
valvular tumors – insufficiency & symptoms of heart failure
Diagnosis - echocardiography and the distinctive fat pattern on MRI.
Require resection.
Lipomatous hypertrophy of the interatrial septum Exaggerated growth of normal fat existing
within the septum and is not a true tumor.
Developmental disorder caused by expansion of adipose tissue trapped in the interatrial septum during embryogenesis
The septal hypertrophy may be as much as 2 cm in thickness and is seen primarily in older patients and in those who are obese
Lipomatous hypertrophy of the interatrial septumAssociated with the presence of CAD in
proportion to the degree of atrial septal thickness
Indistinguishable from lipoma except that the former occurs in the atrial septum with a typical distribution (generally sparing the fossa ovalis).
In the absence of symptoms of atrial arrhythmias,
heart block or rare vena caval obstruction, they do not require resection
Pericardial lipomasIncidental finding and clinically insignificant.
Rarely assumes gigantic proportions and its appearance on a chest radiograph may be mistaken for a huge pericardial effusion or massive cardiomegaly
Benign pericardial lipomas can infiltrate the myocardium. If the ventricular septum is invaded, communication between the pericardial space and the right ventricular cavity may result.
Rhabdomyomas Develop almost exclusively in children, mostly <1
year of age80 to 90% are associated with tuberous sclerosis Usually found in the ventricular walls/AV valves.Most regress spontaneouslyResection is usually not required unless symptomatic Symptoms –due to obstruction of blood flow through
the heart or consist of rhythm disturbancesPresent with features of preexcitation on the ECG
Rhabdomyositis A rare form of cardiomyopathy in infants
Tumor nodules are not grossly apparent
Microscopically, the cardiac muscle fibers and conduction system are diffusely involved with rhabdomyomatous histologic changes
Recurrent atrial tachycardia and sudden death from intractable ventricular tachycardia
Why rhabdomyomas regress?High expression of ubiquitin which starts
expressing from 32 week onwards
Absent mitotic activity
Ubiquitin is responsible for the degradation of rhabdomyoma cells and hence tumor regression
Characteristic spider cells are formed in the process.
Fibromas Second most common pediatric cardiac
tumor and can also occur in adults Histologically similar to fibromas arising
elsewhere in the body. Usually arise in the ventricular muscle and
may become quite large.Do not regress spontaneously. Arise approximately 5 times more frequently
in the LV than RV
FibromasMost common symptom, due to obstruction of
blood flow or interference with valvular function.
Myocardial dysfunction and conduction disturbances
Echocardiography, supplemented with CT/MRI confirms the diagnosis.
Symptomatic tumors should be resected. Complete resection of very large tumors may require cardiac transplantation.
Teratomas Arise within the pericardium, but do not
originate from cardiac structures
Although generally benign,can have serious mechanical consequences by causing tamponade or through direct pressure on the heart.
Risk of death in-utero or immediately after birth
Teratomas Treatment therefore requires either fetal
tumor excision, or caesarean section and immediate operation on the newborn
Have a single blood supply and are not invasive, properly timed tumor surgery is straightforward and successful
Purkinje cell tumors/hamartomas
Consist of small, flat sheets of cells most frequently located in the left ventricle, and on the endocardial and epicardial surfaces
Undetectable by echocardiographic or radiologic techniques.
Tumors of young children and present with incessant ventricular tachycardia
ECGs often demonstrate a bundle branch pattern (right bundle branch block when the tumor is in the left ventricle). Electrophysiologic studies can localize the tumors, facilitating surgical excision.
Paragangliomas Neuroendocrine tumors that can be hormonally
active or inactive. In tumors not producing catecholamines,
symptoms are due to cardiac compression or tamponade.
In contrast, cardiac paragangliomas that are hormonally active primarily produce norepinephrine and may cause systemic symptoms (eg, headache, sweating, tachycardia, hypertension)
Hormonally inactive tumors are more frequent in the pericardium
ParagangliomasMay be localized with echocardiography.
Extremely vascular
Coronary angiography -to plan the operative resection
Paragangliomas Paragangliomas (benign/malignant) within the
pericardium parasitize the cardiac blood supply and hence are very difficult to excise
All intrapericardial paragangliomas require resection
Cardiopulmonary bypass and even circulatory arrest may be required due to the high degree of vascularity, or to moderate the extreme hypertension possible from tumor manipulation or hormonally active tumors
If complete resection is not possible, cardiac transplantation may be required
PRIMARY MALIGNANT TUMORSMalignant tumors constitute approx 15% of
primary cardiac tumors
Sarcomas are the most common
Virtually all types of sarcomas have been reported in the heart
Prognosis depends on Mitotic activity, extent of tumor necrosis and cellular differentiation.
Malignant cardiac tumors
Clinical presentation is largely determined by the location of the tumor, rather than its histopathology.
The diagnostic approach relies upon echocardiography, MRI, and CT to define the presence of a tumor and its anatomic relationship to normal structures
Increased mitoses
Angiosarcomas
Composed of malignant cells that form vascular channels.
Pathology may overlap with Kaposi's sarcoma, which can also involve the myocardium
Arise predominantly in the right atrium
Rhabdomyosarcomas
Constitute as many as 20% of all primary cardiac sarcomas
Most commonly found in adults, although they have also been described in children.
Multiple sites of myocardial involvement are common, No predominant localization within any area of the heart.
Fibrosarcomas
Fibrosarcomas and malignant fibrous histiocytomas are white fleshy ("fish flesh") tumors that are composed of spindle cells, and may have extensive areas of necrosis and hemorrhage
These tumors tend to extensively infiltrate the myocardium
Leimyosarcomas
Spindle-celled, high-grade tumors that arise more frequently in the left atrium
These sarcomas have both a high rate of local recurrence and systemic spread.
Mesothelioma Although most arise in the pleura, these can also
arise from the pericardium, where they are usually malignant
Those arising in the pericardium produce tamponade and constriction
Seen with echocardiography, CT scan, MRI and sometimes by chest x-ray
Pericardiocentesis may yield a cytologic diagnosis
Mesothelioma
More rarely they may arise as benign tumors of the AV node where they may produce heart block
Can be confirmed with echocardiography
Resection is the treatment of choice for mesothelioma, but the prognosis with malignant pericardial mesotheliomas is very poor
The addition of radiation and/or chemotherapy has been attempted but has not been shown to be of value.
Treatment and prognosis In general, sarcomas proliferate rapidly, and
cause death through widespread infiltration of the myocardium, obstruction of blood flow through the heart, and/or distant metastases.
Although complete resection is the treatment of choice, most patients develop recurrent disease and die of their malignancy even if their tumor can be completely resected.
Treatment and prognosisThe median survival is typically 6 to 12 months
although long-term survival has been reported with complete resection.
Low-grade sarcomas may have a better prognosis
Adjuvant chemotherapy and radiation therapy have been used infrequently either after resection to improve results or in metastatic involvement.
Rhabdomyosarcomas may have a better outcome with chemotherapy
Treatment and prognosisThe poor results with surgical resection have
led to occasional attempts to treat patients with cardiac transplantation, if extracardiac disease is not present
cardiac autotransplantation is another novel approach.
Here, the heart is excised, tumor resected ex vivo, and the heart is reconstructed before being reimplanted. The advantage of this procedure is the increased ease with which major resection and reconstruction can be performed, while at the same time avoiding the need for antirejection treatment
Lymphomas and other tumors Primary lymphomas arising in the myocardium have
been reported.
In a review of 40 cases identified from the literature between 1995 and 2002, the outlook was generally poor
However, 38 percent of cases achieved a complete response with systemic therapy.
Other tumors may also arise in the heart, including paragangliomas and extramedullary plasmacytomas
SECONDARY CARDIAC TUMORS
Cardiac involvement may arise from hematogenous metastases, direct invasion from the mediastinum, or tumor growth into the vena cava and extension into the right atrium
Malignant melanomas are particularly likely to metastasize to the heart. Other solid tumors commonly associated with cardiac involvement include lung cancer, breast cancer, soft tissue sarcomas, renal carcinoma, esophageal cancer, hepatocellular carcinoma, and thyroid cancer. There is also a high prevalence of secondary cardiac involvement with leukemia and lymphoma
Secondary cardiac tumorsCardiac or pericardial metastasis should be
considered whenever a patient with known malignancy develops cardiovascular symptoms, particularly if this occurs in conjunction with cardiomegaly, a new or changing heart murmur, electrocardiographic conduction delay, or arrhythmia.
Emboli thought to originate in the heart should also raise the possibility of cardiac involvement with tumor.
Cardiac metastases rarely may be the first manifestation of malignant disease
Secondary cardiac tumors
The specific symptoms reflect the site of cardiac involvement.
The diagnostic evaluation is the same as that for primary cardiac tumors and relies upon echocardiography, MRI, and CT.
Resection of cardiac metastases has been used to provide symptom palliation and prolong life
Summary Cardiac tumours are
rare.A cardiac mass most
likely represents a thrombus or vegetation.
Most cardiac tumours are secondary, ie, originate from a primary tumour elsewhere.
Most primary cardiac tumours are benign
In addition to history, a non- invasive diagnosis of cardiac tumours can usually be made by the following:-Histology based likelihood.-Tumour location.-Age at presentation.-Imaging characteristics
Summary The most common benign
cardiac tumour is a myxoma.
Myxomas are generally found in the left atrium arising from a stalk attached to the fossa ovalis membrane.
The most common primary cardiac tumours in children are rhabdomyomas and fibromas, both of which are benign.
Rhabdomyomas usually decrease in size with age.
The most common cardiac tumour involving valves is a papillary fibroelastoma.
The most common primary malignant tumour is a sarcoma.
Angiosarcomas are the most common histologic subtype of sarcoma and are usually found in the right atrium