Transcript Primary Cardiac Neoplasms

Cardiac Tumor
Seoul National University Hospital
Department of Thoracic & Cardiovascular Surgery
Cardiac Tumor
Definition
Cardiac tumors include benign and malignant neoplasms arising
within the cardiac chambers or in the myocardium.
• History
Columbus : 1st recognition in 1559 followed by Malpighi in 1666,
Morgagni in 1762
Yater : Dissertation & tabulation of primary cardiac tumor in
1931, 1st clinical diagnosis recorded in 1934
Bahnson & Newman : Removal of RA myxoma without CPB
in 1952
Crafoord : Excision of myxoma from left atrium with CPB
Primary Cardiac Tumors
Introduction
• Primary cardiac tumors are uncommon clinical entities
with an incidence of 0.0017% to 0.03%
• The majority of these tumors are benign atrial
myxomas, successfully managed by surgical excision
• Malignant cardiac tumors continue to present a
difficult therapeutic challenge, and surgical resection is
often necessary to alleviate the severe symptoms
associated with these tumors, but is, nevertheless,
associated with poor long-term prognosis
• Because of the rarity of primary cardiac malignancies,
therapeutic concepts and methods of surgical resection
have not been standardized.
Primary Cardiac Tumors
Types
• Benign ; approximately 70%
Myxoma 29.3%, Lipoma 10.1%, Primary fibroelastoma 9.5%,
Rhabdomyoma 8.1%, Fibroma 3.8%, Hemangioma 3.4%,
Teratoma 3.2%, Mesothelioma of AV node 2.7%,
Granular cell tumor, Neurofibroma, Lymphangioma
• Malignant ; approximately 30%
Angiosarcoma 8.8%, Rhabdomyosarcoma 5.8%,
Mesothelioma 4.2%, Fibrosarcoma 3.2%, lymphoma 1.6%,
Extraskeletal osteosarcoma 1.1%, Neurogenic sarcoma,
Malignant teratoma, Leiomyosarcoma, Liposarcoma,
Synovial sarcoma
Primary Cardiac Neoplasms
Clinical features
1. Incidence
0.001~0.03% with an autopsy
2. Nature
Benign ; 70 – 75%
myxoma (1/2), lipoma , papillary fibroelastoma,
rhabdomyoma, etc
Malignant ; 25 - 30%
sarcomas (3/4), mesothelioma, lymphoma
3. Symptoms
Embolism, intracardiac obstruction, infiltration
constitutional symptoms (CHF, palpitation)
Cardiac Myxoma
Definition
* Primary cardiac tumors that are generally pedunculated, polypoid
but may have a broad base.
* The cells are uniform, small, and polygonal, with round or oval
nuclei and a moderate amount of cytoplasm of myxoid matrix of
acid mucopolysaccharide.
* They lie in a myxomatous stroma in which elements are seen and
covered by endothelium and endothelium-lined crevices and clefts.
• Morphology
Myxomas are intracavitary tumors occurring within any of the
cardiac chamber, but they have a predilection for the atria and
particularly the left atrium, usually 5 to 6 cm in diameter
Cardiac Myxoma
Nature
• Increasing numbers of reports indicate the malignant
potential.
Extensive local invasion has been noted.
• Nearly all solitary myxoma have a normal DNA ploidy
(chromosomal pattern), and nearly all nonfamilial.
• Multiple myxomas usually have an abnormal DNA ploidy
pattern, are usually familial in their occurrence, and tend
to recur.
• In rare circumstances, atrial myxomas may become
infected.
Cardiac Myxoma
Location
• Atrial myxoma
* Majority of atrial myxoma, whether left or right, arise from the
atrial septum (90%), usually from limbus of fossa ovalis.
* 80 – 90% of myxomas are in the left atrium.
• Ventricular myxoma
Found mainly on the right ventricular free wall or ventricular
septum, sometimes described as infiltrating
• Valvar myoxoma
Rare reports arising from tricuspid, mitral, and pulmonary valve
Cardiac Myxoma
Pathophysiology
• Hemodynamic derangement
Obstruct pulmonary or systemic venous drainage or impair flow across AV
valve progressively. When such obstruction is intermittent, syncope, often
related postural change, or sudden death.
• Embolism
Systemic emboli occur in 30 to 45% with left atrial myxoma.
Embolism from right-sided tumor occurs in 10%, may cause fatal pulmonary
obstruction, or pulmonary hypertension.
• Constitutional manifestations
A plethora of constitutional symptoms and laboratory findings may be only the
manifestations and these occur in 30%.
Fever, weight loss, clubbing, Raynaud’s phenomenon, myalgia, arthralgia,
unusually polycythemia, hemolytic anemia, thrombocytopenia.
Immune reaction to neoplasm(elevated globulin) result in constitutional
symptoms and elevated ESR & C-reactive protein.
Cardiac Myxoma
Patterns
• Familial
1. 5% of patients, Mandelian dominant inheritance
2. Primarily young men, less commonly (62%) in left atrium,
more often multiple(33%), 20 % of patients associated with other
unusual conditions (Cushing, pituitary tumor, Sertoli cell tumor
of testis, centrofacial and labial lentigenosis, cutaneous myxoma,
multiple mammary fibroadenoma )
3. Strong tendency to recurrence
4. Abnormal DNA ploidy pattern
• Nonfamilial (sporadic)
1. Primarily of middle aged women
2. Usually single (94%), in left atrium (75%), uncommonly associated
conditions and uncommonly recur.
3. Only about 20% have abnormal DNA ploidy pattern
Cardiac Myxoma
Natural history
• Incidence
Prevalent in older adult, 2-3 times more common
in female, rare in child
• Natural course
* Highly variable
* Once symptoms of dyspnea and hemoptysis develop
in left atrial myxoma, or symptoms of abdominal
protuberance from ascites or hepatomegaly in
right atrial myxoma, rapidly progress to death
within 1 – 2 years onset.
Cardiac Myxoma
Surgical treatment
• Indication
Indicated whenever a diagnosis of cardiac myxoma is
made on urgent basis
• Technique
Atrial myxoma ; sufficient excision of atrial septum if
possible, uninvolved tissue 5 mm
beyond
Other myxoma ; ventricular myxoma does not require
excision of full-thickness ventricular
wall
Cardiac Myxoma
Surgical results
• Survival
Early death ; uncommon
Time-related survival ; uncommon except recurrence
• Recurrence
Sporadic ; unusual, only 1-3%
Familial ; 30-70% recurrence, probably related, 40% of
patients with abnormal DNA ploidy pattern
• Postoperative arrhythmias
Atrial arrhythmias are common
• Functional status ; generally good
Papillary Fibroelastoma
Etiology
• There are several hypotheses about the etiology
of cardiac papillary fibroelastoma
• Fibroblasts infiltrate and proliferate within mural
thrombi replacing them gradually with fibrous
tissue
• Cytomegalovirus infection may underlie the
development of cardiac papillary fibroelastoma
• Concurrent hypertrophic obstructive
cardiomyopathy, usually characterized by
myocyte hypertrophy and disarray, and interstitial
and endocardial fibrosis; histologic changes that
may predispose to the development of cardiac
papillary fibroelastoma.
Papillary Fibroelastoma
Characteristics
• Definition
Benign tumors about 7% of cardiac tumor, curable and
identifiable cause of strokes and other embolic events
• Morphology
Papillary fronds, usually small, but extensively involves.
Usually develop on a valve leaflet, most commonly aortic
or mitral leaflet.
• Clinical features
Responsible for embolism by detachment of fronds or
thrombus formation on its surface
Papillary Fibroelastoma
Clinical features
• Natural history
Nothing appears to be known.
Tendency to produce emboli
• Technique of operation
Excision with preservation of valve
Valve replacement in multicentric or extensive involvement
• Results
No recurrence is reported.
• Indications
Removal is indicated, embolic events or incidental finding
Rhabdomyoma
Introduction
• Definition
Histologically specific cardiac tumors tend to occur in
tuberous sclerosis, same as, cardiac hamartoma, Purkinje
cell tumor, histiocytoid cardiomyopathy.
• Morphology
1. Yellow-gray tumor, round or polygonal, spider cell
containing a central cytoplasmic mass suspended by
fine, fibrillar processes radiating periphery, considered
altered myocytes.
2. Invariably occur in the cardiac ventricles, with both side
equally, and very commonly in multiple location in the
heart.
Rhabdomyoma
Characteristics
• Clinical features
Most common primary cardiac tumors in children
May occur in siblings
Presentation is frequent at birth or few days of life with CHF
Incessant ventricular tachycardia, atrial arrhythmias in infants
Later in a child with tuberous sclerosis, mental retardation,
seizures, occasionally
• Natural History
Death nearly occurs in such patients in infancy or children
• Results
Generally unsatisfactory, a few without tuberous sclerosis
obtain good result, but ½ of these develop tuberous sclerosis.
Rhabdomyoma
Operative treatment
• Surgical indications
When the tumor is solitary and flow is obstructed,
operation is indicated.
Contraindicated in the presence of tuberous sclerosis &
mental retardation with seizure, especially multiple
tumors and no symptoms.
• Technique of operation
Excision of mass protruded in ventricular cavity
Excision is limited to the area of tumor
Rhabdomyoma
Cardiac Fibroma
Introduction
• Definition
Benign tumors, grossly resembling uterine leiomyoma with
a whorled appearance on cut section, microscopically, consist
of elongated fibroblasts mixed with collagen and elastic fibers
• Morphology
Exclusively within ventricular myocardium and frequently in
the ventricular septum, usually bulky tumors, not infiltrating
• Clinical features
Varied presentation, cardiac murmur in infancy and
cardiomegaly
Cardiac Fibroma
Clinical features
• Natural history
Rare tumor, its natural history is unknown
Most fibromas diagnosed in infancy & childhood, &
eventually cause death by obstructing ventricular
inflow or outflow.
• Technique of operation
Enucleated by blunt and sharp dissection, and easily
separated from the surrounding myocardium
• Indication of operation
The diagnosis in a symptomatic patients
Cardiac
Fibroma
(Gross)
Cardiac
Fibroma
(Microscopy)
Malignant Cardiac Tumors
Introduction
• Malignant tumors continue to present a therapeutic
challenge as incomplete resections universally result in
rapid local tumor recurrence
• Resection of left atrial tumors include radical resections
using left atrial approaches through the interatrial
groove or transseptally through the right atrium.
• For left ventricular tumors, surgical resection can be
accomplished through a transaortic valve approach, a
ventriculotomy, or a transmitral valve approach.
• Cardiac autotransplantation is a technique with a novel
application for resection of malignant cardiac tumors
• Orthotopic heart transplantation and combined heartlung transplantation have been previously described
Angiosarcoma
Secondary Cardiac Tumor
Introduction
• Secondary tumors in the heart are 20 to 40
times more common than primary cardiac
lesions
• Common malignancies that involve the heart
include the breasts, lungs, lymphoma,
melanomas, and sarcomas.
• The order of frequency of involvement is
pericardium, epicardium, myocardium, and
endocardium .
Secondary Cardiac Tumor
Symptoms
• Cardiac metastasis rarely is solitary.
• Symptoms by cardiac metastasis are
produced in only 10% of patients.
• Most of the symptoms produced are by
pericardial effusion and tamponade ,
refractory arrhythmia , or congestive
heart failure.
Ventricular Outpouchings
Introduction
• Congenital ventricular outpouchings include
congenital ventricular diverticulum (CVD) and
aneurysm (CVA)
• Although various characteristics have been
proposed to differentiate the two entities,
definition remains incomplete.
• In addition, their embryologic origin, clinical
profile, natural course, and treatment are
unclear since these malformations are rare
Ventricular Outpouchings
Definition
• CVD was characterized by synchronous systolic contractility,
which was correlated to the presence of muscular fibers within the
diverticular wall on histologic examination.
• When apical, CVD was a fingerlike contractile pouch with narrow
connection to the ventricle ands a part of the Cantrell syndrome
• When nonapical, CVD was a large contractile pouch with wide
connection to the ventricle and was never associated with
intracardiac or extracardiac abnormalities.
• In contrast, CVA was a large akinetic or dyskinetic pouch with
wide connection to the ventricle and endocardial or transmural
fibrosis on histologic examination.
• CVA was never associated with intracardiac or extracardiac
defects.
Ventricular Outpouchings
Etiology
• Apical CVD is a part of the Cantrell syndrome and has
a common defect in embryologic development with
midline thoracoabdominal formation
• The development of nonapical CVD and CVA has been
attributed to a focal defect of the muscular ventricular
wall due to an intrinsic abnormality in embryogenesis.
• CVA has also been suspected to be acquired in the
prenatal period, potentially as a result of a viral
infection or coronary lesions including stenosis,
hypoplasia, and localized intimal proliferation.
Ventricular Outpouchings
Prognosis and outcome
• The prognosis for apical CVD depended on associated
intracardiac malformations and was generally good
after repair.
• The outcome for nonapical CVD was also excellent,
with even a total regression in some cases
• CVA had a poor prognosis with frequent fatal
cardiovascular complications in the neonatal period.
Usually died of heart failure within a few days after
birth.
Poor prognostic factors such as size and progression of
the CVA and signs of cardiac failure.
Ventricular Outpouchings
Treatment
• When symptomatic or when associated with other
cardiac abnormalities, surgical treatment is usually
recommended.
• Controversy concerns whether an asymptomatic and
isolated CVD should be treated surgically.
• CVD can potentially give rise to embolism, arrhythmias,
cardiac failure, and rupture
• A different approach be considered for asymptomatic
CVA with severe endocardial fibrosis or transmural
fibrosis because paradoxical contractions cause
dilatation and expose the aneurysm to the risk of
rupture.