4. INTRODUCTION
• Primary heart tumours are quite rare, and found
in 0.04% of autopsies
• The 5 most common ones are benign and
collectively, accounting for 50% to90% of
primary tumours of the heart
• The heart is also a site for metastatic tumours in
about 5% of persons dying of cancer
5. • In descending order of frequency, the benign
tumours encountered in the heart are:
myxoma, lipomas, fibroelastomas,
rhabdomyoma, haemangioma and
lymphangioma
• The malignant tumours are still rarer, the
important ones are: rhabdomyosarcoma,
angiosarcoma and malignant mesothelial
6. • The diagnosis of primary cardiac tumours is
frequently challenging
• The symptoms associated with most primary
cardiac tumours are nonspecific, and this
elusiveness often results in a delay in the
diagnosis of disease
7. CLINICAL MANIFESTATION
• It can be divided into four general categories:
1. Systemic manifestation
2. Embolic manifestation
3. Cardiac manifestation
4. Phenomena secondary to metastatic
diseases
8. MYXOMAS
• The most common primary tumour of adult heart
comprising about 50% of all primary cardiac tumours
• Majority occur in the age range of 30 to 60 years.
• A benign lesion thought to arise from primitive
multipotent mesenchymal cells
• It may be located in cardiac chamber or valves, but
90% of them are situated in the left atrium
9. MORPHOLOGY
• Usually single tumour, but rarely can be multiple
• Favoured site of origin is the fossa ovalis in the atrial septum
• Ranges from <1cm to ≥ 10cm
• It can be sessile or pedunculate lesions
• It varies from globular hard masses mottled with haemorrhage or soft,
translucent, papillary or villous lesion having gelatinous appearance
resembling an organizing mural thrombus
• Some investigators actually consider them to be organising mural thrombi
rather than true neoplasm
10. • The pedunculate form often move during systole into the
atrioventricular valve opening, resulting in position
dependent intermittent obstruction
• Sometimes mobile tumour damage valve leaflets as it
exert a “wrecking-ball” effect
• Histologically, stellate or globular myxoma cells are
embedded within an abundant acid mucopolysaccharide
ground substance
• Haemorrhage and mononuclear inflammation are usually
present
11. • Microscopically, the tumour shows the following features:
i) There is abundant myxoid or mucoid intercellular stroma positive
for mucin
ii) The cellularity is sparse. The tumour cells are generally stellate-
shaped, spindled and polyhedral, scattered in the stroma. Occasional
multinucleate tumour giant cells are present
iii) Numerous capillary-sized blood vessels are found and the tumour
cells may aggregate around them
• iv) A few lymphocytes, plasma cells and macrophages are seen
• v) Foci of haemorrhage and deposits of haemosiderin granules are
often present
15. CLINICAL MANIFESTATION
• Often results from valvular “ball-valve” obstruction,
embolization, or constitutional symptoms, like fever and
malaise (due to cytokines elaboration by some myxomas
e.g IL-6 a major mediator of acute-phase response
• At times there can be fragmentation and systemic
embolisation
• Echocardiography identifies the mass
• Surgical removal is curative; however with incomplete
excision, recurrence can happen months to years later
16. LIPOMAS
• Affect both myocardium and pericardium
• It can reach several centimetres in size
17. LIPOMA
• It is a localised, well circumscribed, benign tumour
composed of mature fats cells
• It can occur in the subendocardium, subepicardium, or
myocardium
• Asymptomatic, or produce ball-valve obstructions or
arrhythmias
• Location is often in the left ventricle, right atrium, or atrial
septum
18. PAPILLARY
FIBROELASTOMA
• Usually incidental, lesions most often identified at
autopsy
• They may embolism and then become clinically
important including stroke
• Clonal cytogenetic abnormalities, suggests it as
unusual benign neoplasms
• It may resemble remotely organised thrombus on
aortic valves of older individuals
19. MORPHOLOGY
• >80% are located on valves, particularly the ventricular
surfaces of semilunar valves and atrial surfaces of
atrioventricular valves
• Each lesion, typically 1 to 2cm in diameter, consists of a
distinctive cluster of hairlike projections up to 1cm in
length giving a Sea anemone-like appearance
• The projections are covered by a surface endothelium
surrounding a core of myxoid connective tissue with
abundant mucopolysaccharide matrix and elastic fibers
20. An autopsy specimen that
shows an incidentally found
papillary fibroelastoma
It appears as a small rounded
multilobulated mass attached to
the edge of aortic valve cusp on
the ventricular side of the
valve(black arrow)
23. FIBROMAS
• Encapsulated, solitary tumours
• Frequently in the septal myocardium
• often encroach on the conduction system as they
grow
• With septal involvement, ventricular fibrillation is
often the first presentation
• Resection of septal fibroma is not possible
24. RHABDOMYOMA
• Most frequent primary tumour of the paediatric heart
• Commonly discovered within the first years of life due to obstruction of a
valvular orifice or cardiac chamber
• Half of the cases are due to sporadic mutations; other 50% often
associated with tuberous sclerosis, mutations in tumour suppressor gene
TSC1 or TSC2
• TSC1 and TSC2 proteins (hamartin and tubers) function in a complex that
inhibits a kinase mammalian target or rapamycin (mTOR), that stimulate
and regulates cell size
• They are considered as hamartomas rather than true neoplasms as they
often regress spontaneously
25. MORPHOLOGY
• It appears as grey-white myocardial masses
• It may be small or up to several cm in diameter
• They are usually multiple and involve the ventricles
preferentially, protruding into the lumen
• It consist of bizarre, markedly enlarged myocytes
microscopically
• The abundant cytoplasms is often artifactually reduced to thin
strands that stretch from the nucleus to the surface membrane
during routine histologic processing , this appearance is referred
to as “spider” cells
26. • A large firm, white
tumour mass was
found filling the left
ventricle
• This is a cardiac
rhabdomyoma
28. ANGIOSARCOMA
• Most common primary malignancy of the heart
• Malignant cells that form vascular channels
• Right atrium commonly involved
• There is pericardial involvement with haemorrhage effusion and
thrombus
• Diffuse, irregularly shaped
• Mean survival is one year
• Successful treatment with chemotherapy and radiotherapy followed by
transplant has been reported
29. RHABDOMYOSARCOMA
• Most commonly seen in adults
• No chamber selectivity
• No pericardial involvement
• Multiple sites of cardiac involvement
• Poor prognosis
• Limited success with resection and adjuvant treatment
31. MESOTHELIOMAS
• Diffuse pericardial tumour
• Involved both parietal and visceral pericardium
• Superficially invade the myocardium
• Rarely invade the cardiac chambers
• Pericarditis or tamponade
• Poor prognosis
• Temporary improvement with radiotherapy or chemotherapy
32.
33. SECONDARY TUMOURS
• Metastatic tumours of the heart are more
common than the primary tumours
• About 10% cases with disseminated cancer
have metastases in the heart
• Most of these results from haematogenous
or lymphatic spread
34. • In descending order of frequency, primary
sites of origin are: carcinoma of the lung,
breast, malignant lymphoma, leukaemia and
malignant melanoma
• Occasionally, there may be direct extension
of a primary intrathoracic tumour such as
carcinoma of the lung into the pericardium
and into the cardiac chambers
Atrial myxoma. A, A large pedunculated lesion arises from the region of the fossa ovalis and extends into the mitral valve orifice. B, Abundant amorphous extracellular matrix contains scattered multinucleate myxoma cells (arrowheads) in various groupings, including abnormal vessel-like forma- tions (arrow).
