Cardiac Tumours Flashcards
Characteristics of benign tumours?
- Generally not harmful
- Well-differentiated cells
- Slow growing
- Well-circumscibed or encapsulated
- Non-invasive, non-infiltrative
- Do not metastasise
Characteristics of malignant tumours?
- Harmful
- Poorly or undifferentiated cells
- Rapid growing
- Poorly circumscribed or not encapsulated; irregular shape
- Locally invasive, infiltrative
- Frequently metastasise
- Primitive appearing, unspecialised
Echo appearance of benign tumours?
- Well-circumscribed or encapsulated
- Freely mobile in relation to adjacent structures
- Do not invade or infiltrate surrounding normal tissues
Echo appearance of malignant tumours?
- Poorly circumscribed, irregular in shape
- Not encapsulated
- Fixed to adjacent structures
- Locally invasive, infiltrating surrounding tissue
Characteristics of primary tumours?
- Originate in the heart
- Can be benign or malignant
Characteristics of secondary tumours?
- Metastasised to the heart
- Malignant
- Secondary metastatic malignant disease more common than primary
What are the 4 pathways of metastatic spread of tumours to the heart?
- Lymphatic
- Haematogenous
- Direct extension
- Venous extension
What is lymphatic spread of tumours to the heart?
Tumour cells invade into lymphatic capillaries and travel to heart via lymph channels
What is haematogenous spread of tumours to the heart?
Spread through blood; invasion of tumour into local capillaries
What is direct extension of tumours to the heart?
Often arises via organs close to the heart in which cause tumour grows into the heart itself
What is venous extension of tumours to the heart?
Tumour invades large systemic vein and migrates to involve right heart by way of the vena cava
Example of direct extension tumour?
SCC of lung:
- Direct extension into LA via pulmonary veins
Example of venous spread tumour?
Renal cell carcinoma:
- IVC and RA extension; finger like mass extending through IVC into RA
- Can track mass back to kidney to confirm origin
Example of lymphatic spread tumour?
Pericardial tumour:
- Echo-genetic mass in pericardium
- Can result in pericardial constriction
Example of haematogenous spread tumour?
Carcinoid syndrome:
1. Carcinoid TV Disease
- Appearance of thickened and retracted leaflets that remain in fixed semi-open position throughout cardiac cycle
- Severe TR as a result
2. Secondary Cardiac Carcinoid Tumour
- Rarely, carcinoid tumour may metastasise to the heart resulting in an intra-myocardial carcinoid metastasis
Most common primary benign and primary malignant cardiac tumour in adults?
- Benign: myxoma
- Malignant: angiosarcoma
Most common primary benign and primary malignant cardiac tumour in children?
- Benign: rhabdomyoma
- Malignant: rhabdomyosarcoma
% of cardiac tumours that are benign/malignant?
90% benign
10% malignant
Most common location for cardiac myxomas?
- LA attached to IAS at fossa ovalis
- Less common in RA, ventricles
Morphological features of cardiac myxomas?
- Usually solitary
- Ovoid and gelatinous; regular smooth surface
- Attached via a narrow base (pedicle/stalk)
- Range in size from 1-15cm (av. 5-6cm)
- Calcification, fibrosis, haemorrhage, necrosis is common
Cardiac myxomas and functional stenosis?
LA myxomas usually quite mobile and may prolapse through MV causing functional mitral stenosis
What are the myxoma variants?
- Multi-lobulated
- Liquefaction (echo-luscent area in myxoma representing hematoma/liquefaction)
- Calcification (echo-bright areas within myxoma representing calcification)
What is Carney Complex?
- Familial myxomas
- Autosomal dominant syndrome comprising multiple and recurrent myxomas of the heart and skin, hyperpigmentation of the skin and endocrine dysfunction
What is the most common tumour of cardiac valves?
Papillary fibroelastoma (PFE)
Most common location of papillary fibroelastomas?
- Aortic and mitral valves
- Less common on pulmonary and tricuspid valves
Morphological features of papillary fibroelastoma?
- Usually solitary
- Small gelatinous polyps with multiple fronds
- Rarely exceeds 1cm diameter
- Attached to endocardium by a short pedicle or stalk
Echo appearance of papillary fibroelastoma?
- Small, round homogenous masses attached to valve
- May appear highly mobile with independent motion - “pom-pom” appearance
What is a lipoma?
Benign tumours of ‘fat’ cells
Most common location for lipomas?
Sub-epicardial (larger), sub-endocardial, intra-myocardial
Morphological features of lipoma?
- Homogenous fatty encapsulated tumours
- Broad based
- Variable size (larger in sub-epicardium)
Echo appearance of lipoma?
Echo-bright, homogenous appearance
What is lipomatous hypertrophy of IAS?
- Non-neoplastic condition (may be mistaken for cardiac tumour)
- Result of accumulation of excess adipose tissue within IAS with sparing of fossa ovals membrane
- Sparring of fossa ovals membrane leading to dumbbell appearance
What is a rhabdomyoma?
- Benign tumour of striated muscle
- Most common cardiac tumour in children
Most common location of rhabdomyomas?
LV and/or RV myocardium; outflow tracts; AV valves (MV and TV)
Morphological features of rhabdomyomas?
- Frequently multiple
- White-grey to yellow, flesh lesions
- Well-circumscribed, non-infiltrative
- Range in size: few mm to several cm
- Spontaneous regression in size, number or both is common
What is Tuberous Sclerosis?
- Rare, multi-system genetic disease (autosomal dominant)
- Characterised by formation of hamartomas in multiple organs
- Diagnostic criteria based on major and minor criteria; cardiac rhabdomyomas major criteria for diagnosis of tuberous sclerosis
Echo appearance of rhabdomyomas?
Acoustic properties similar to myocardium
What are cardiac fibromas?
- Benign connective tissue tumours
- Second most common cardiac tumours in children
Most common location of cardiac fibromas?
LV and ventricular septum
Morphological features of cardiac fibromas?
- Usually solitary
- Firm, white, and well-circumscribed
- Tightly adherent to adjacent myocardium
- Almost always intramural
- Range in size from 1-10cm
- Do not regress spontaneously
Echo appearance of cardiac fibroma?
Can mimic hypertrophy cardiomyopathy or septal thickening
Characteristics of primary cardiac sarcomas?
- Account for 95% of malignant primary cardiac tumours
- Fast growing with poor prognosis
- Death through widespread infiltration of myocardium, obstruction of blood flow, and/or distant metastases
- Show different histologies
- Classified based on underlying nature of the tumour
Characteristics of angiosarcomas?
- Malignant vascular tumours
- Most common malignant cardiac tumour in adults
- Most common location = right heart (esp. RA)
Characteristics of rhabdomyosarcomas?
- Malignant tumours of striated muscle
- Most common malignant cardiac tumour in children
- Multiple sites of myocardium
Characteristics of fibrosarcomas?
- Malignant tumour of fibrous connective tissue
- Often involving more than 1 cardiac chamber
Characteristics of leiomyosarcomas?
- Malignant tumours of smooth muscles
- Rarely seen in heart
- Usually found within atria
Characteristics of osteosarcoma?
- Malignant tumours of the bone
- May be intramural or intra-cavity
- Usually originate in posterior LA wall near pulmonary veins
Characteristics of primary cardiac lymphoma?
- Rare (5% of all primary malignant cardiac tumours)
- More common in immunosuppressed adults
- Most commonly involves right heart and pericardium; less common in left heart and IVS
Most common type of tumour in the LA?
Myxoma (70-80% of cases)
Most common type of tumour on valves?
Papillary fibroelastoma (> 90% of cases; L > R)
Most common type of tumour in the RA?
Angiosarcoma (80 - 90% of cases)
Most common type of tumour in the ventricles?
- Fibroma (> 95% of cases)
- Rhabdomyoma (60% of cases)
Appearance of highly vascular, malignant tumours following contrast?
Greater enhancement compared with surrounding myocardium
Appearance of tumours with poor blood supply (e.g. myxomas) following contrast?
Appear hypoenhanced compared with surrounding myocardium
Appearance of thrombus following contrast?
No enhancement as thrombus is avascular (thrombus appears black, LV cavity illuminated by bubbles)
Appearance of LV lymphomas following contrast?
Lymphomas are highly vascular = complete enhancement of mass
Appearance of LA myxoma with contrast?
Myxomas have poor blood supply = partial enhancement of mass
Other appropriate imaging modalities to assess cardiac tumours?
- TTE, TOE, CMR and CT all considered appropriate
- Cardiac CT and CMR are often combines with TTE
