Dr.P.V.Nishanth

lntroductionRepresent an important group of

cardiovascular abnormalities because early and accurate diagnosis may be curative and sometimes avoids unnecessary surgery.

frequency of only 0.001-0.03%

• About 75 percent of all primary cardiac tumors -benign neoplasms.

• Remaining 25 percent of primary cardiac tumors -malignant neoplasms-metastatic MC

CLINICAL PRESENTATIONFour general categories—Systemic manifestationsEmbolic manifestationsCardiac manifestationsPhenomena secondary to metastatic diseases.

Systemic Manifestations:Produced by secretory products released by

the tumor and/or by tumor necrosisConstitutional symptoms of fever, chills,

fatigue, malaise, and weight loss.Leukocytosis, polycythemia/ anemia, throm-

bocytosis/ thrombocytopenia, hypergammaglobulinemia, and increased ESR

Mimic those of several connective tissue diseases

Embolic PhenomenaSystemic emboli-typically by a left-sided

tumorRight-sided tumors - concurrent right-to-left

shunting through a patent foramen ovale. Brain -MC site -involvement of both

hemispheres and multiple regions is seen more than 40 percent of the time

• Cerebral embolism -transient ischemic attack or an ischemic stroke, but lCH may occur as well.

• Mild vertigo to seizure and even a comatose state.

• Delayed aneurysm formation presumably at the site of previous cerebral tumor emboli

• Tumor emboli to a coronary artery-myocardial infarction

• Pulmonary embolization is typically caused by a right-sided tumor

Benign- cardiac myxomas are most frequently associated with embolic findings, especially when the tumor possesses a villous surface

Other benign primary cardiac neoplasms that are known to produce emboli –

Papillary fibroelastomas hemangiomas/lymphangiomasMalignant tumors can embolise

Cardiac ManifestationsDirect mechanical interference with

myocardial/valvular function lnterruption of coronary blood flow lnterference with electrophysiological

conductionStimulation of pericardial fluid accumulation

Intramural or myocardial – asymptomatic, especially if the sizes are small.

Located within or pressing on major cardiac conduction pathways -complete heart block or asystole in more severe cases

Compress the cardiac cavitiesObstruct the ventricular outflow tractContribute to insufficiency of the mitral valve

lntracavitary• Left atrial-can interfere with the mitral valve • Signs & symptoms-sudden in onset, intermittent,

and positional-• Fatigue, dyspnea, orthopnea, PND, chest pain,

pulmonary edema, and peripheral edema• S3 loud and widely split S1• Holosystolic murmur most prominent at the apex

with radiation to the axilla, • Diastolic murmur from turbulent blood flow

through the mitral orifice• Tumor plop -the tumor striking the endocardial

wall or the abrupt halt of tumor excursions occurs later than an opening snap but earlier than an S3.

Right atrium-right heart failureOften delayed with an average time interval from

presentation to the correct diagnosis of 3 yearsRapidly progressive right heart failure and also

new-onset heart murmurs because of mechanical interference with the tricuspid valve by the tumor

Elevated JVP with prominent a-wave and steep y descent, and an early diastolic murmur or holosystolic murmur

SVC syndrome - findings of peripheral edema, HSM, ascites,

• Right ventricular tumors –• Intracavitary component may obstruct the

filling or the outflow of the RV –RHF• Auscultation may reveal a systolic ejection

murmur at the left sternal border, an S3, and a delayed P2.

• An elevated JVP and Kussmaul sign may also be present.

• These findings may vary significantly depending on the position of the patient

Left ventricular tumors obstruct the LVOT and produce findings of LVF

and syncope, as well as atypical chest pain from obstruction of a coronary artery either by direct tumor involvement or tumor emboli.

Metastatic DiseasesLate stage with systemic dissemination

present.Present with symptoms secondary to the

metastatic diseaseCommon sites of metastases -lung, brain, and

bone, although metastases to other sites reported.

DIAGNOSTIC APPROACH

AetiologyCan often be determined by considering four

factors: (1) Histology based likelihood (2) Age of the patient at time of presentation (3) Tumor location (4) Non-invasive tissue characterisation

Histology based likelihood• 90% of primary cardiac tumours excised

surgically are benign, with nearly 80% of these tumours representing myxomas

• Papillary fibroelastomas (26%)• Fibromas (6%)• Lipomas (4%)• Calcified amorphous tumours,

haemangiomas, teratomas, unilocular developmental cysts, and rhabdomyomas

10% of primary cardiac tumours excised at surgery are malignant, of which more than 90% are sarcomas.

The remaining few are represented by lymphomas

Age at presentation

Location

Non-invasive tissue characterisationEchocardiography:echogenicity of the mass and whether

calcification is present. Vascularity can also be assessed using colour

flow Doppler and echocardiographic contrast. Strain imaging also has potential in

identifying the non-contractile nature of masses such as fibromas

CT- regarding vascularity by contrast enhancement, presence of calcification, and presence of fat.

MRI also provides information regarding vascularity, presence of fat, degree of tissue oedema, and possibly iron content

Primary benign cardiac tumoursCardiac myxoma30-50% of all benign tumors¸25% of all

tumors and cysts of heartUsually solitary and develop in the atria, 75%

originating in the left atrium and 15-20% in the right atrium

Arise from or near the interatrial septum at the border of the fossa ovalis membrane

Other sites-post.wall ant.wall appendageRA myxoma-broader base larger areaVentricular-free wall/IVS sessile/pedunculatedMultiple- 5% biatrial common.

Occur in all age groups, most frequently between the third and sixth decades

Women are more commonly affectedMyxomas usually occur sporadically,

Familial- 7%-Carneys complex Younger age, MultipleAtypical locations.Increased risk of recurrence after resectionOther features- spotty skin pigmentation,

endocrine hyperactivity and other tumors such as testicular Sertoli cell tumor, psammomatous melanotic schwannoma, pituitary adenoma, and thyroid .

mutations in PRKAR1A, a regulatory subunit 1A of cAMP-dependent protein kinase A

Morphology • Range in size from 1-15 cm in diameter. • Polypoid with a smooth or gently lobulated

surface, often pedunculated, and characteristically arise from a narrow stalk.

• Polypoid myxomas rarely embolise• Villous or papillary myxomas have multiple

fine fragile villous extensions -greatest risk of embolisation.

Clinical featuresDetermined by their location, size, and

mobility.One or more features of the triad of embolism,

intracardiac obstruction, and constitutional symptoms.

Left atrial-Dysnea /Fatigue MR-pounding of valve by tumorPostural variationSymptoms out of sync with mitral valve

diseaseUnexplained emboli

RA-RHFD/D of RHFVentricular-sessile Obs. Less common Emboli-left -64% RT-10%-PAH/ recurrent PE

X rayEvidence of elevated left atrial pressure -53

percent of patients with left atrial myxoma Cardiomegaly is seen in 37 and 50 percent of

left and right atrial myxomas, respectively. Intracardiac tumor calcification is a rare

finding in left atrial myxomas but is found in 56 percent of patients with right atrial myxoma

ECHOAppear as homogenous echo masses Echo free spaces-hemorrhageAreas of calcfcaton

M-mode

LA-tumor fills LA in systole Diastole-prolapses into mitral valve orifice

mass of echoes appear behind AMLEF slope decreases

• CE CT - overall attenuation lower than that of myocardium

• CMR shows heterogeneous signal intensity in 90 percent of cardiac myxomas,

• T1- images - isointense signal• Cine gradient-echo CMR - superior to other

imaging modalities

Histologically-sparsely distributed uniform spindle- and stellate-shaped cells within an extensive myxoid stroma.

Generally hypocellularstroma and the tumor cells stain positive with

PAS, whereas only the stroma shows positive staining with Alcian blue

D/DVegetation-leaflets /clinical settingMural thrombi-setting-MS/DCM appendage laminated immoble broad base

Treatment Surgical en bloc resection with a margin of

normal tissue, if anatomically feasible, is considered curative and is the treatment of choice

Overall risk of recurrence after resection is 13%, but is much more common with familial myxomas than with sporadic tumours (22% vs 3%).

Hazard of recurrence increases linearly for 4 years after resection, after which risk of recurrence is low.

Based on this observation, semi-annual surveillance echocardiographic follow-up has been recommended for 4 years following surgery

• Immediate postoperative mortality in most series ranges from 0 to 7.5 percent.

• Common postoperative complications include arrhythmias

• Recurrence of myxoma in the brain-• Growth of the embolized tumor fragments, • Difficult to manage, but chemotherapy is not

recommended because embolic myxomas do not truly represent meta-static diseases.

• Rare but potentially life-threatening complication is the development of cerebral aneurysm secondary to embolic tumor fragments.

Papillary fibroelastomaMC from valvular endocardium10% of primary cardiac tumours Second most common primary cardiac

tumourAbove 60 yrs of ageVentricular surface of semilunar valves and

atrial surface of AV valvesAdults-aortic valve ( 37 to 45 percent)Children-tricuspid valve.

Characteristic flower-like appearance with multiple papillary fronds attached to the endocardium by a short pedicle, -typical ‘sea anemone’ appearance when immersed in saline

Usually solitary (91 percent) and <1 cm in diameter but can be larger, particularly when they occur in the cardiac chambers

• Often asymptomatic.• MC -systemic embolisation resulting from

attached thrombi as well as from fragmentation of the papillary fronds themselves -50% of symptomatic patients

• Rarely, patients present with subacute bacterial endocarditis–like findings, and pulmonary embolism and sudden death have also been reported.

Men and women are equally affected. There is a strong association with

hypertrophic obstructive cardiomyopathy (HOCM), as well as surgical, radiation, and haemodynamic trauma

Echo- usually appearing as a small, mobile, pedunculated valvular mass.

They usually have a well defined ‘head’ and characteristically have a stippled edge with a ‘shimmer’ or ‘vibration’ at the tumour blood interface

TEE - definitive imaging modality

CT/MRI- temporal and spatial resolution is often still inadequate to characterise most of these tumours since they are usually small

Preoperatively CT angiograms -coronary vasculature, in order to avoid manipulating the tumours into the coronary ostia at the time of coronary angiography.

Lipoma and lipomatous hypertrophy• <5% of surgically excised primary cardiac

tumours• MC site-LV/RA• Usually arise from the epicardial surface,

most often from a broad pedicle, and grow into the pericardial space.

• Subendocardial lipomas are often small and sessile grow as broad based pedunculated masses protruding into the cardiac chambers.

• Asymptomatic but may cause symptoms due to local compression or arrhythmias

• More echo dense than myxomaMRI -diagnostic – superior in differentiating

between fat and connective tissue- high in signal intensity on T1 and show evidence of signal dropout on fat saturation sequences

Lipomatous hypertrophy of the atrial septum

may often be confused with a cardiac tumour. It is a benign non-neoplastic condition that

results from adipose cell hyperplasia and is associated with increasing age and obesity.

It involves the limbus of the fossa ovalis, sparing the fossa ovalis membrane and resulting in a characteristic dumbbell shape

Interatrial septum -diameter exceeding 2 cm in transverse dimension

Does not cause any symptomsIn rare instances in which the tumor

protrudes into the right atrium and the superior vena cava, patients can present with symptoms secondary to blood flow obstruction

• CT and CMR –• IAS is thickened to up to 7 cm, whereas

normally it is less than 1 cm• LHAS with symptomatic arrhythmias can be

managed medically, whereas surgical excision should be restricted to the rare cases in which the disease causes symptomatic hemodynamic obstruction

RhabdomyomaMC in children (80 percent younger than the

age of 1) most common cardiac mass in childhood -

50% to 75% of pediatric cardiac tumorsCommonly associated with tuberous

sclerosis- predilection for the ventricles, and they often involve the interventricular septum.

1 to 3 cm in size/multiple.

Sixty percent of the older children and less than 25% of adults with tuberous sclerosis, however, will have detectable cardiac masses

Occasional cases are seen in the absence of tuberous sclerosis-Approximately 50% of the lesions are single

Often do not demonstrate spontaneous regression, and they may require surgery

The lesion typically appears as a yellow-gray, firm, circumscribed lobulated mass, and the size of the tumor can range from less than 1 mm to 9 cm

Fetal/Infant Adult

Result in stillbirth or early postnatal death - significant hemodynamic impairment.

Obstruction may occur to either the RV/LVOT-prominent intracavitary component, and significant cardiac murmurs

Can regress spontaneously

Always assoc. wth tuberous sclerosis

most common –arrhythmias

SporadicSpontaneous regression

rare

• high incidence of ventricular pre-excitation and Wolff Parkinson White syndrome, and may increase the risk of arrhythmia

• A characteristic and peculiar feature of rhabdomyomas is spontaneous regression in size or number or both in most patients <4 years of age

Appear well circumscribed and slightly brighter than the surrounding normal myocardium.

Appear hypodense on contrast CT- isointense to myocardium on T1 weighted images and hyperintense on T2 weighted images.

.

As spontaneous tumour resolution is common, management is expectant in asymptomatic patients

Occasionally, surgical resection is necessary if the tumours are large, resulting in structural or haemodynamic complications.

Cardiac fibromaIt is the most common resected cardiac

neoplasm in children and the second most common benign primary cardiac tumour found at autopsy in children

characteristically solitary (unlike rhabdomyomas) and are invariably located in the ventricles

Ventricular septum/ the LV free wall/ the right ventricle/ the atria in that order

Typically well circumscribed, and often centrally calcified without cystic change, necrosis or haemorrhage.

They usually affect children, a third of whom are younger than 1 year at presentation.

Most cardiac fibromas appear to occur sporadically

Gorlin syndrome- basal cell carcinomas of the skin, odontogenic keratocysts, rib and vertebral anomalies, and multiple skin lesions

One third of patients present with arrhythmias, one third with heart failure or cyanosis, and one third are detected incidentally.

Less common presenting findings include sudden death and atypical chest pain

ECG –LVH/ RVH/ BBB /AV block/ VTXray- cardiomegaly with or without focal

bulge, and calcification -15 percent of cases

Echo-discrete often obstructive, echogenic, noncontractile mass ranging in size from 1-10 cm in diameter in a ventricular wall.

The tumour may mimic hypertrophic cardiomyopathy or ventricular septal hypertrophy

• CT-homogenous masses with soft tissue attenuation that may be either infiltrative or sharply marginated.

• Calcification is often seen. • MRI-homogeneous and hypointense on T2

weighted images and isointense relative to muscle on T1 weighted images.

• Little or no contrast material enhancement.• MRI also demonstrates the extent of

myocardial infiltration which can guide tumour resection.

• Surgery appears to be the optimal treatment in patients with symptomatic resectable tumours.

• The role of surgery in patients with asymptomatic tumours is less clear, as cardiac fibromas can remain dormant for many years and even regress.

• However, because of fatal arrhythmias, surgery is often recommended despite absence of symptoms.

• Transplantation is considered for large and unresectable tumours

Hemangiomas and Lymphangiomas

Less than 2 percent of primary cardiac neoplasms

Occur in any age group ranging from a few months to the seventh decade of life

• Clinical presentation of is variable• Arrhythmias

• CHF

• pericardial effusion• Ventricular outflow tract obstruction• Giant cardiac hemangioma can result in

Kasabach-Merritt syndrome -thrombosis, consumptive thrombocytopenia, and coagulopathy.

• Occasionally be associated with hemangioma in extracardiac sites

• Echo-sensitive -cardiac hemangioma appearing typically as a hyperechoic lesion.

• CAG-can sometimes demonstrate blood supply to the tumor, with the presence of “tumor blush

• Chest CT- heterogeneous signal with intense enhancement in most cases after contrast material administration.

• On CMR-with intermediate signal intensity on T1-weighted images and hypointense signal on T2-weighted images and there may be rapid enhancement during contrast infusion

Radical resection -recommended because of the potential for recurrence, especially if the resection is incomplete

The postoperative prognosis is excellent in resectable cases

Conservative management may be considered in asymptomatic patients, particularly if complex and potentially hazardous excision is required

Malignant primary cardiac tumours• Exceedingly rare.• 15% of primary cardiac tumours • Vast majority (95 per cent) – sarcomas• 5%- primary cardiac lymphomas and

mesotheliomas• secondary cardiac malignancy- 30 times more

common-lung and breast cancer.

General features High mitotic activity (>5 mitotic figures/10 high-

power fields), extensive tumor necrosis, and poor cellular differentiation presence of metastases - poorer prognosis.

CT or CMR - large, heterogeneous, broad-based masses that frequently occupy most of the affected cardiac chambers

Sarcoma• 3rd & 5th decades of life• M=F• Commonly affect the left side, mostly the left

atrium• Rapidly progressive with a median survival of

1 year due to widespread local infiltration, intracavitary obstruction

• Metastases-often already present at the time of initial presentation

• Angiosarcomas-• 30 to 37 percent of the cases90 percent -right atrium(differentiating

feature in that most of the other sarcomas have a left atrial predilection,)

• Dyspnea, chest pain, heart murmur, constitutional symptoms, arrhythmias, superior vena cava syndrome, and evidence of congestive heart failure.

• pericardial effusion and cardiac tamponade• metastatic disease –stroke like neurologic

symptoms secondary to cerebral metastases

• Echocardiography – broad based right atrial mass near the inferior vena cava.

• CTand MRI - avid, arterial phase enhancement permitting a definitive diagnosis.

• Transvenous echo-guided cardiac biopsy/biopsy of the metastatic lesion in a more accessible location or cytology examination on pericardiocentesis fluid

• Novel lymphatic endothelial markers including D2-40

Treatment• Mean survival of 9 to 10 mon. -late detection

of the disease—most patients present with advanced-stage disease.

• Integrated approach -combination of surgery, irradiation, adjuvant/neoadjuvant chemotherapy, and immunotherapy using interleukin-12 (IL-12).

• Advanced-stage unresectable disease, palliative treatment including -metallic stents for SVC syndrome and for severe RVOTO

Rhabdomyosarcomas• Most common primary sarcoma of the heart in

children• Average age of disease presentation is in the

second decade of life• M>F• Multiple lesions are frequently present (60

percent).• Embryonal type and pleomorphic type of -

primary tumors in the heart • Alveolar type - metastatic disease to the heart.

congestive heart failure, arrhythmias, cardiac murmurs, and constitutional symptoms

Nonspecific ECG and chest radiography findings are often present.

TTE/TEE guided biopsy -attempted for tissue diagnosis, a negative result cannot be relied on because there is a high rate of false negatives

Chest CT or CMR -delineation of the nature, origin, and extent of the lesion, especially if a malignant lesion is suspected

• Metastases-MC to the lung and lymph nodes, • Survival is usually less than 1 year. • High risk biopsy and extensive myocardial

and pericardial extension are associated with the worst prognosis.

• Highly infiltrative nature of tumor often precludes surgery.

• Tumor has a poor response to radiation and chemotherapy

• Heart transplant -if no obvious distant metastases are present

Leiomyosarcomas• Mean age of presentation is in the fourth

decade, and there is no apparent sex predilection.

• Dyspnea, pericardial effusions, chest pain, atrial arrhythmias, and congestive heart failure.

• 70 to 80 percent -the left atrium, and they tend to extend into the pulmonary trunk.

• Typically solitary but can be multiple in 30 percent of patients

Prognosis is poor with a mean survival of 6 months after diagnosis.

Because of the tendency of leiomyosarcomas to recur, cardiac transplantation is not a realistic option

LYMPHOMAS1.3 to 2 percent of all primary cardiac tumorsImmunocompromised individuals - more

commonPTLD -chronic immunosuppression and

Epstein-Barr virus infection. Lymphomas -HIV and PTLD -extracardiac

involvement at presentation, and isolated cardiac involvement is rare.

• Right side of the heart in 69 to 72 percent of the cases

• Single lesion in 66 percent and multiple lesions in 34 percent of the cases.

• 80 percent -immunocompetent individuals -diffuse large cell B-cell lymphoma type

• Immunocompromised patients- small noncleaved or immunoblastic lymphomas

• 62 to 67 years with a range of 13 to 90 years• M>F• chest pain, congestive heart failure, pericardial

effusion, palpitation, and arrhythmias Frequently involve the epicardium and extend

to involve the pericardium• TEE- excellent for initial visualization • CT and CMR are superior at delineating the

infiltrative nature of the tumor and CMR has the highest sensitivity for detecting primary cardiac lymphomas

• Sensitive to chemotherapy - anthracycline-based chemotherapy with or without radiation therapy -mainstay of treatment for primary cardiac lymphomas

• Radical surgical excision is generally discouraged

Metastatic disease of the heart and pericardium20 to 40 times more common than primary

cardiac neoplasiametastatic melanoma-46% to 64%lung carcinoma-36%leukemia, lymphoma-20%, carcinoma of the breast 7%,carcinoma of the esophagus 6%

Frequent presence of pericardial effusions, unexplained shortness of breath, and the new development of an arrhythmia in a patient with a known malignancy

Four pathwaysDirect extension-lung carcinomas, as well as

primary mediastinal tumors such as malignant thymoma

Retrograde extension-lymphatics-lung and esophageal carcinoma-pericardium frequently is involved

Hematogenous spread -melanoma, sarcomas, leukemia, and renal cell carcinoma –manifested most often by multiple intramyocardial metastatic deposits

Transvenous extension to the right side of theheart -renal cell carcinoma, adrenal carcinoma, or hepatocellular carcinoma

Transvenous extension to the left atrium is seen most often in primary lung carcinoma

ConclusionsCardiac tumours are being increasingly

recognised antemortem, permitting earlier diagnosis and treatment

Aetiology can often be determined by considering the histology based likelihood, the age of the patient at time of presentation, tumour location and non invasive imaging.

CT and MRI are complimentary techniques, often better suited for intramyocardial and pericardial lesions as well as for assessment of extracardiac spread.

For benign cardiac tumours, an early diagnosis and appropriate treatment is not only possible but often curative.

Unfortunately the outcome for malignant primary tumours, even despite early diagnosis and aggressive treatment, remains dismal.