Cardiac Tumors Flashcards
Cardiac Tumors — epidemiology?
~25% are malignant
~75% are benign
myxoma (~50%) > metastasis (~20%) > lipoma (~10%) > papillary fibroelastoma (~10%)
Cardiac Tumors — features suggesting malignancy?
Invasion of extra-cardiac structures
Involvement of more than one cardiac chamber
Involvement of the right side of the heart
Tissue inhomogeneity
poor definition of borders
greater than 5cm diameter
presence of pericardial or pleural effusion
myxoma — epidemiological facts?
~50% of primary cardiac tumors in adulthood
more common in females — 3:1 (f:m)
7% with atypical behaviour — familial predisposition or as part of the Carney complex
Carney complex — facts?
autosomal dominant inheritance syndrome — myxomas, hyperpigmented skin lesions, extracardiac tumors (pituitary adenomas, breast fibroadenomas, melanotic schwannomas)
myxoma — appearance / MRI diagnosis?
well defined, spherical, intracavitary mass lobular contours, inhomogeneous ~25% broad-based ~75% pedunculated Cine-SSFP: mobile T1w: isointense to myocardium T2w: hyperintense CE+ less than myocardium
myxoma — clinical symptoms?
Symptoms are often unspecific
myxoma — complications?
obstruction
arrhythmia
embolic events
interference with mitral valve
myxoma — differential diagnosis (DDx)?
thrombus lipoma papillary fibroelastoma endocarditic vegetations prominent crista terminalis
myxoma — therapy?
surgical removal
cardiac lipoma - definition ?
benign cardiac neoplasia
deriving from neoplastic proliferative adipocytes
cardiac lipoma — epidemiology?
2nd most common cardiac neoplasia in adulthood ~10%
equally common in female and male
myxoma — localisation?
~75% left atrium (LA) mostly atrial septum, fossa ovalis
~20% right atrium
~5% ventricles
cardiac lipoma — appearance / MRI diagnosis?
mostly homogeneous encapsulated tumors sometimes septa T1w: hyperintense T2w: hyperintense no contrast enhancement (CE-) FS: hypointense
cardiac lipoma — localisation?
~50% subendocardial
~25% intramyocardial
~25% subepicardial
most often located in the right atrium
cardiac lipoma — clinical symptoms?
mostly asymptomatic
cardiac lipoma — complications?
arrhythmia
embolic events
cardiac lipoma — therapy?
case-dependant
surgical removal
cardiac lipoma — differential diagnosis (DDx) ?
thrombi
myxoma
papillary fibroelastoma
lipomatous hypertrophy of the atrial septum
lipomatous hypertrophy of the atrial septum — facts?
characterised by fatty infiltration of the atrial septum sparing of fossa ovalis prevalence estimated 1-8% incidence increasing with age brown fat cells
papillary fibroelastoma — epidemiology?
3rd most common primary cardiac tumor in adulthood
~10% of all primary cardiac tumors
~70% of all primary heart valve tumors
peak age ~6th decade
papillary fibroelastoma — appearance / MRI diagnosis?
usually < 2cm in size pedunculated, sea-anemone like T1w: hypointense T2w: hyperintense CE-
papillary fibroelastoma — localisation ?
~90% on heart valves
~29% aortic, ~25% mitral, ~17% tricuspid, ~13% pulmonary valve
~10% somewhere else on endocard
papillary fibroelastoma — clinical symptoms ?
mostly asymptomatic
dependant on localisation
papillary fibroelastoma — complications ?
obstruction (coronary ostium) embolic events (eg TIA) also paradoxical with PFO
papillary fibroelastoma — aetiology ?
multiple different theories (harmatoma / thrombus)
can develop after cardiac surgery
associated with endothelial injury
most probable mechanical irritation
papillary fibroelastoma — differential diagnosis (DDx) ?
thrombi
endocarditic vegetations
valvular myxoma
papillary fibroelastoma — therapy ?
case dependant
surgical removal
cardiac fibroma — what is it ?
harmatoma, consisting of fibroblasts, collagen and elastic fibres
cardiac fibroma — epidemiology ?
2nd most common primary cardiac tumor in childhood
~10% of all primary cardiac tumors in childhood
~15% of cardiac fibromas are diagnosed in adulthood
cardiac fibroma — appearance and MRI diagnosis ?
intramural
calcifications in ~25%
Cine-SSFP: isointense to myocardium
T1w: isointense to myocardium
T2w: mildly hyperintense in childhood, hypointense with increasing age and in adulthood (specific)
no enhancement in first pass perfusion
peripheral enhancement and hypointense core on LGE
cardiac fibroma — localisation ?
left ventricular free wall > right ventricular free wall > septum
cardiac fibroma — clinical symptoms ?
dependant on localisation and implications
arrhythmia
outflow obstruction
ischemia
cardiac fibroma — complications ?
arrhythmia
ischemia
outflow obstruction
sudden death
cardiac fibroma — therapy ?
case dependant
asymptomatic — no therapy because of complicated surgery
symptomatic — surgical removal
cardiac fibroma — prognosis?
no tendency of spontaneous regression
increase of collagen fibres with age (T2w increasingly hypointense)
septal localisation, large size, young age have worse prognosis
cardiac fibroma — differential diagnosis (DDx) ?
cardiac rhabdomyoma hypertrophic cardiomyopathy (HCM) hemangioma
cardiac hemangioma — facts ?
non-neoplastic venous malformation
~50% cavernous
cardiac hemangioma — epidemiology ?
~3% of all cardiac tumors
cardiac hemangioma — appearance, MRI diagnosis ?
T1w: isointense
T2w: hyperintense, homogeneous
fast wash in in first pass perfusion
cardiac hemangioma — clinical symptoms ?
variable
dependant on size, localisation and implications
cardiac hemangioma — complications ?
arrhythmia
pericardial effusion / haemorrhage
cerebral apoplex
cardiac hemangioma — therapy ?
case dependant
surgical resection
cardiac hemangioma — differential diagnosis (DDx) ?
all cardiac tumors
rhabomyoma
cardiac lymphoma — facts ?
primary cardiac lymphoma
non Hodgkin lymphoma (NHL), B-cell lymphoma
associated with immunosuppression
cardiac lymphoma — epidemiology ?
~1.3% of all cardiac tumors
age peak of~60 years
cardiac lymphoma — appearance, MRI diagnosis ?
T1w: isointense
T2w: hyperintense
CE+
diffusion restriction
cardiac lymphoma — clinical symptoms ?
B-symptoms
fatigue, weight loss, night-sweats
cardiac lymphoma — complications ?
pericardial effusion
pericardial tamponade
heart failure
arrhythmia
cardiac lymphoma — therapy ?
chemotherapy: Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, Prednisolone (R-CHOP)
autologous stem cell transplant
cardiac lymphoma — differential diagnosis (DDx) ?
sarcoma
amyloidosis
sarcoidosis
cardiac angiosarcoma — epidemiology ?
most common primary malignant cardiac tumor ~30%
male > female — 2-3:1
peak age 4th decade
cardiac angiosarcoma — localisation ?
right atrium (RA) most common two types: intracavitary growth infiltration of the pericard
cardiac angiosarcoma — appearance, MRI diagnosis ?
two types: intracavitary growth, infiltration of the pericard
T1w: isointense with hyperintense spots and flow voids
T2w: hyperintense
CE+
cardiac angiosarcoma — clinical symptoms ?
unspecific
dependant on subtype
cardiac angiosarcoma — complications ?
right heart failure
pericardial effusion / haemorrhage / tamponade
cardiac angiosarcoma — therapy ?
multimodal
cardiac angiosarcoma — differential diagnosis (DDx) ?
- all malignant cardiac tumors
- cardiac lymphoma (respects more anatomical structures)
- Erdheim-Chester-disease (respects anatomical structures)
cardiac rhabdomyosarcoma — epidemiology ?
most common malignant cardiac tumor in childhood
~20% of all primary malignant cardiac tumors
cardiac rhabdomyosarcoma — appearance, MRI diagnosis ?
infiltrative growth necrotic areas often multiple locations T1w: isointense T2w: hyperintense CE+ inhomogenous enhancement
cardiac rhabdomyosarcoma — clinical symptoms & complications ?
unspecific symptoms: fatigue, weight loss
dependant on localisation: outflow obstruction
pericardial effusion
cardiac rhabdomyosarcoma — therapy and prognosis ?
Therapy : surgical resection — often not entirely possible
Prognosis : often death within a year
cardiac metastasis — epidemiology ?
2nd most common cardiac tumor after myxoma
up to 40 times more common than primary malignant cardiac tumors
in 3-18% of all patients with malignancies
absolutely most frequent in breast and lung cancer
relatively most frequent in melanoma
cardiac metastasis — appearance, MRI diagnosis ?
intramural or intracavitary often multiple T1w: hypo or isointense T2w: hyperintense CE+ T1 hyperintense, T2 hypointense (melanoma)
cardiac metastasis — localisation ?
RV>LV>RA>LA
cardiac metastasis — therapy
usually chemotherapy because of late stage
cardiac metastasis — differential diagnosis (DDx) ?
myxoma
thrombi
primary malignant cardiac tumors
cardiac thrombus — epidemiology ?
most frequent intracavitary tumor like lesion
incidence between 3-21% depending on age
cardiac thrombus — aetiology and pathophysiology ?
intracavitary areas with low blood flow or stasis
atrial fibrillation, mitral stenosis
myocardial infarction, left ventricular aneurysm
cardiomyopathy
cardiac thrombus — localisation / predilection sites ?
left atrial appendage — atrial fibrillation, mitral stenosis
left ventricular apex — myocardial infarction, aneurysm
cardiac thrombus — diagnosis ?
dependant on localisation
left atrium / left atrial appendage — TEE (CT)
left ventricle — MRI (CT)
cardiac thrombus — MRI findings ?
early LGE: intracavitary filling defect
acute thrombi: T1w and T2w intermediary
subacute thrombi: T1w hyperintense, T2w hypointense (methemoglobin)
chronic thrombi: T1w and T2w hypointense (fibrous remodeling)
cardiac thrombus — complications ?
embolic events
~15% of ischaemic cerebral infarcts
cardiac thrombus — therapy ?
anticoagulation
left atrial clipping
Cardiac masses — role of MRI ?
I1 Indication
- confirmation of presence and localisation of cardiac tumors
- assessment of tumour size and locoregional extension
- mass characterisation (T1w, T2w, T1wCE, EGE, LGE)
- assessment of haemodynamic effect
- surgical therapy planning
Cardiac masses - assessment ?
- confirmation of a mass / tumour
- location and anatomical relationship
- size
- form and margins
- motion and functional significance
- tissue characteristics (T1, T2, CE)
- tumour associated complications
- features suggesting malignancy
- differentiation
Cardiac masses - indicators of benignancy ?
- origin in left atrium
- single lesion
- smooth margins
- encapsulated lesion
- endocavitary growth
Cardiac masses - non-neoplastic origin ?
- normal or hypertrophic cardiac structures (e.g. crista terminalis)
- echocardiographic artefacts
- thrombi
- intraparietal haematoma
- pseudoaneurysms
- granulomatous disease
- focal hypertrophic cardiomyopathy
Cardiac paraganglioma - facts ?
- T2w hyperintense
- rich vascular supply
- LA>RA>aortic bulb
- catecholamine production
- DDx: cardiac hemangioma
