Cardiac Tumors and Their Management

1. Cardiac Tumors
P re s e n t e d B y ： S a m i m A z i z i
H a m a d M e d i c a l C o r p o ra t i o n
C a r d i o t h o ra c i c S u r g e r y D e p a r t m e n t

2. CONTENTS
Classification
Management
Epidemiology
Clinical
Manifestations
Conclusion

3. 1
Classification

4. Benign Malignant
Primary Secondary
1- Classification

5. 第 5 页
1 - Classification
Primary Benign Tumors

6. 第 6 页
1 - Classification
Primary Malignant Tumors

7. 第 7 页
1 - Classification
Secondary Tumors
Cardiac Metastases ( Tumors Metastatic to the Heart)
• 40x more common than primary tumors
• Malignant melanoma – highest predilection for cardiac metastasis (50-65%)
• Most common from breast and lung CA
• Almost always occur in the setting of widespread primary disease

8. 2
Epidemiology

9. 第 9 页
2 – Epidemiology
Incidence of Benign Tumors

10. 第 10 页
2 – Epidemiology
Incidence of Malignant Tumors

11. 3
Clinical Manifestations

12. In general, the clinical manifestations of cardiac
tumors can be divided into four mechanistic
categories:
1. Systemic manifestations
2. Embolic manifestations
3. Cardiac manifestations
4. If Malignant >> Phenomena secondary to
metastatic diseases
3- Clinical Manifestations

13. 3- Clinical Manifestations
1- Systemic Manifestations:
• Constitutional symptoms of fever, chills, fatigue, malaise, and weight loss.
• These systemic manifestations are believed to be produced by secretory
products released by the tumor or by tumor necrosis.
• Most commonly seen in : Cardiac Myxoma Caused by Increased Serum
IL-6 levels.

14. 3- Clinical Manifestations
2- Embolic Manifestations:
• Brain: Most common site: TIA , Stroke , ICH
• Coronary artery embolism: MI
• Pulmonary Embolism: if Right sided or L-R Shunt present

15. 3- Clinical Manifestations
3- Cardiac Manifestations:
• Direct mechanical interference with myocardial or valvular function
• Interruption of coronary blood flow
• Interference with electrophysiologic conduction
• Accumulation of pericardial fluid
• Left Atrial >> Mitral Regurgitation And/Or Mitral Stenosis

16. Benign Tumors:
Myxoma: (Mostly LA Mass)
• Most common type of primary cardiac tumor (1/3 to ½ of all cases)
• Most commonly in 3rd – 6th decade; female > male
• Sporadic vs familial
• Majority sporadic; some are familial (autosomal dominant transmission)
3- Clinical Manifestations

17. 3- Clinical Manifestations
Clinical Presentation:
• Systemic Findings
Cardiovascular findings:
1. Atrial: Symptoms resemble mitral valve disease
• Stenosis – Tumor prolapse into the mitral orifice during diastole
• Regurgitation – Injury to the valve by tumor-induced trauma
2. Ventricular – Outflow obstruction > syncope

18. 3- Clinical Manifestations

19. 3- Clinical Manifestations
Rhabdomyomas: (Mostly in Ventricles)
• Most common in infants and children (75% < 1 y/o)
• Symptoms due to mechanical obstruction, mimic valvular stenosis, CHF, restrictive or
hypertrophic cardiomyopathy
• Multiple in 90% of cases
• May be associated with tuberous sclerosis, adenoma sebaceum, benign kidney tumors

20. 3- Clinical Manifestations- Malignant
Malignant Tumors
Sarcoma: (Most Commonly in RA and Pericardium)
• Most common malignant tumor; 2nd most common primary tumor of heart
• Common in male (3:1)
• Characterized by rapidly downhill course leading to patient’s death weeks to
months from time of presentation due to:
1. Hemodynamic compromise
2. Local invasion
3. Distant metastases

21. 3- Clinical Manifestations- Malignant
Histologic types:
1. Angiosarcomas – most common
2. Rhabdomyosarcoma
3. Fibrosarcoma
4. Osteosarcoma
• Characterized by rapid growth
• At presentation, often spread extensively for surgical excision
• Right sided failure, pericardial disease, vena cava obstruction
• May occur in left side > mistaken for myxoma

22. 3- Clinical Manifestations- Malignant
Treatment & Prognosis:
• Surgery not curative > to establish diagnosis
• Occurrence of distant metastases

23. 3- Clinical Manifestations- Malignant
Metastatic Tumors:
• 40x more common than primary tumors
• Malignant melanoma – highest predilection for cardiac metastasis (50-65%)
• Most common from breast and lung CA
Location:
1. Pericardium – most common
2. Myocardium
3. Rarely, endocardium and cardiac valves

24. 3- Clinical Manifestations- Malignant
Clinical presentation:
1. Dyspnea – most common
2. Signs of pericarditis
a) Chest pain aggravated by coughing, inspiration or recumbency
b) Pericardial friction rub on auscultation
c) Characteristic ECG changes
3. Cardiac tamponade
a) Increased JVP
b) Pulsus paradoxus
c) Echo evidence of RA and RV collapse

25. 3- Clinical Manifestations- Malignant
Malignant Carcinoid:
• Tumors that elaborate vasoactive amines (eg serotonin), kinins, indoles
(diarrhea, flushing, labile BP)
• Gastrointestinal carcinoids
1- Almost exclusively in the right side
2- Occur only with hepatic metastases
3- Substance responsible for the cardiac lesions inactivated by passage
through liver and lungs
• Lesion: fibrous plaques on the endothelium of cardiac chambers, valves, and
great vessels > result in distortion of the cardiac valves

26. 3- Clinical Manifestations- Malignant
Malignant Carcinoid Clinical syndrome:
1. Tricuspid regurgitation, pulmonic stenosis or both
2. High-output cardiac state may occur – due to decrease in systemic
vascular resistance
3. Coronary artery spasm due to a circulating vasoactive substance

27. 4
Management

28. 4- Management
Benign Tumors: (75% 5-years survival)
Severe obstruction or intractable arrhythmias >> immediate resection (if possible)
The long-term results for resected benign tumors are excellent, whereas
sarcomas have shown a dismal prognosis with few long-term survivors
Vander et al. 2000

29. 4- Management
Malignant Tumors:
• Multiple studies have reported a median survival of 6 months after surgical
resection
• Usually large and deep before detection (i.e. fibrous skeleton involved)
This makes complete resection impossible
• An alternative treatment, cardiac auto-transplantation, has shown promise. In
these cases, the heart is excised, the tumor is resected ex vivo, and the heart is
reconstructed before being reimplanted.
• The advantage of this procedure is the increased ease with which major resection
and reconstruction can be performed, while at the same time avoiding the need for
antirejection treatment Reardon et al. 2006

30. 4- Management
Burke et al. reported that among 40 patients with sarcoma who underwent surgery:
• The overall survival rate in their series was poor, with a mean of 11 months and
a median of 6 months
• They suggested that adjuvant chemoradiation might improve survival
• Left-sided tumors, a low mitotic index and the absence of metastases were
associated with a more favorable outcome
• Age, gender, the presence of differentiation and histological type had no impact
on prognosis
Burke et al. 1992

31. 4- Management
Bakaeen et al. reported:
• A median survival of 9.6 months for patients with malignant disease in a
series of 85 patients with cardiac tumors (80% benign, 20% malignant).
Bakaeen et al. 2003

32. 4- Management
Vitovskii et al. reported that in a series of 33 patients with malignant cardiac
tumors who underwent radical surgical resection:
• Extensive procedures such as resection and repair of the interatrial septum and
atrial walls and valve replacement did not improve survival.
Vitovskii et al.

33. 4- Management
Renal cell tumors:
A 5 year survival rate of 75% has been achieved after nephrectomy with
resection of tumor extension into the vena cava and the right atrium
Adjuvant radiation or chemotherapy can be beneficial in palliating patients’
symptoms and improving quality of life
Shahian et al.

34. 4- Management
Creating a large pericardial window into the left pleural space using the VATS
approach is indicated in some patients to prevent pericardial tamponade
Caccavale et al.

35. 4- Management
Heart transplantation :
Michler et al. published a series of 28 patients who underwent orthotopic heart
transplantation for primary cardiac tumours (21 malignant tumours). The
mean survival for patients with primary cardiac malignancy was 12 months.
Although technically feasible, considering the small number of donor organs
and the extensive recipient list of patients without cancer, the morbidity and
mortality involved with immunosuppression as well as the potential effect of
immunosuppression on any remaining malignancy, heart transplantation is
not recommended for these patients.
Michler et al.

36. 4- Management
Surgical approach:
• Median Sternotomy is usually preferred
• The venae cavae are cannulated either through the RA or
directly
• Bi-atrial incision may be necessary to expose the tumor at
the site of its attachment.
• For right atrial tumors, both venae cavae may be cannulated
directly.
• Whether blood removed from the field during tumor
manipulation should be discarded or returned to the pump
circuit is controversial.
Attum et al.
Read et al.

37. 4- Management
Evolving therapeutic approaches:
• Translocation in chromosomes 12 and 15 (t(12;15)
(p13q25)): New tyrosine kinase inhibitors
• MDM-2 gene is often over-expressed
(MDM-2 binds to and inhibits p53 activity)
• Induction of apoptosis in cancer cells is another evolving
therapy option. However, considering the effect of
apoptosis on cardiac function leading to Heart failure.

38. 5
Conclusion

39. 5- Conclusion
Published studies on primary cardiac tumors are relatively
limited because of the rare occurrence of this tumor type.
Therefore, the diagnosis of cardiac tumors necessitates a
high level of suspicion.
Surgery remains the cornerstone in the therapy of cardiac
tumors and it should be attempted once it is technically
feasible.
The role of adjuvant and neoadjuvant chemoradiation
requires further investigation.

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