cardiac tumors.pptx

morphological characters of cardiac tumors simplified

1. Cardiac Tumors Dr.Aliaa Shaban Lecturer of Cardiovascular Medicine Tanta University

2. Primary & secondary

3. Clinical manifestations: Systemic – constitutional (fever, arthralgias, weight loss, fatigue) and paraneoplastic syndromes (primary cardiac tumors ). Embolic – pulmonary and/or systemic by tumor / thrombo emboli. Cardiac – mass effect interfering with myocardial function , blood flow, electric impulse formation and conduction, pericardial fluid dynamics. Secondary to metastatic disease of a primary malignant cardiac tumor – manifestations depending on site of metastasis[

4. Dyspnea Chest pain

5. Benign cardiac tumors Myxoma LIPOMA Papillary fibroelastoma

6. Myxoma

7. Myxoma Myxomas are the most common benign cardiac tumors (50%) usually affect middle-aged women . Most cases are sporadic. familial myxomas, also known as “syndrome myxoma” or “Carney complex”, being rare (10%). This syndrome is characterised by multiple, recurrent cardiac myxomas at younger age, associated with spotty skin pigmentation and endocrine over-reactivity .

8. Clinical manifestations of myxomas consist in a triad: 1- constitutional symptoms, 2- embolization 3- intracardiac obstruction.n (mitral stenosis), and or incompetence (injury of the valve) Location – left atrium (75%), right atrium (23%), rarely in the ventricles; usually connected to the atrial septum ( fosa ovalis) by a narrow stalk; depiction of site and type of tumor attachment is important when differentiating from thrombus 

9. Lipoma the second most common benign cardiac tumors (10-20%). They represent encapsulated fatty accumulations, are variable in size and usually asymptomatic. Sometimes they may cause symptoms related to compression, arrhythmias and conduction abnormalities. Location – anywhere in the heart, most frequently in the left ventricle, right atrium, and atrial septum. They arise most commonly in the subepicardium and subendocardium , but intramyocardial lipomas have also been described. Subepicardial lipomas often grow into the pericardial space resulting in effusions and subendocardial lipomas might protrude in the cardiac chambers, interfering with blood flow.

10. Lipomatous hypertrophy  of the atrial septum is a benign cardiac mass resulting from fatty infiltration of the atrial septum. Its described incidence on echocardiographic studies is 8% and, typically, is associated with increasing age and obesity.

11. Papillary fibroelastoma Papillary fibroelastomas are the most frequent tumours involving the heart valves. They account for approximately 8% of primary benign cardiac tumors and are usually encountered in older ages. Due to their embolic potential, papillary fibroelastomas (particularly left-sided) should be considered for resection even if asymptomatic . Location – aortic and mitral valves, less often, tricuspid and pulmonary valves or mural endocardium. Different from vegetations, papillary fibroelastomas are located on the downstream side of the valve and rarely cause valvular dysfunction

12. Rhabdomyomas Rhabdomyomas are the most common primary cardiac tumors in infants and young children. approximately three thirds occurring in patients less than 1 year old. There is a strong association between cardiac rhabdomyomas and tuberous sclerosis (80%). In more than half of the patients they regress in size and number after infancy. …..The presence of rhabdomyomas in adulthood is uncommon. Rhabdomyomas most often occur in the right and left ventricular myocardium. On echocardiography they appear as multiple, homogeneous and hyperechogenic tumors located intramurally or pedunculated, protruding into the ventricular cavity, and ranging from few mm to few cm in size. Depending on their location and size they may induce arrhythmias or blood flow obstruction. Symptomatic rhabdomyomas bear a poor prognosis and surgery is necessary, even though complete resection is rarely achieved due to their deep intramyocardial location ,

13. Fibroma Fibromas are the second most common primary cardiac tumors in children. In adults they account for approximately 3% of all benign cardiac tumors . Site: the ventricular myocardium, usually in the left ventricular free wall or interventricular septum. They may mimic hypertrophic cardiomyopathy. Number: Fibromas appear as well circumscribed, highly echogenic, solitary tumors (different from rhabdomyomas), often extending into the ventricular cavity. Typically, their size ranges from 1 to 10 cm and have central calcifications . Symptoms: n 70% of cases, fibromas cause symptoms by impeding intracavitary blood flow or causing ventricular arrhythmias. Given the increased risk for fatal arrhythmias and sudden death, associated with this type of tumors , surgical resection i s recommended regardless symptoms

14. Synopsis…. Fibroma Solitary Ventricular Ventricular Arrhythmia Surgery

15. Hemangioma benign vascular tumors accounting for 2% of primary cardiac tumors . They can occur at any age and are occasionally associated with similar tumors in the gastrointestinal tract or skin. Hemangiomas can occur in any cardiac chamber, but most often are located in the ventricles, growing intramurally or towards the ventricular cavity. Similarly to fibromas, they can mimic hypertrophic cardiomyopathy. Hemangiomas appear as hyperechogenic, well demarcated masses, ranging from 1 to 8 cm in size. In 30% of cases multiple tumors are described. In symptomatic patients radical surgical resection is indicated. Periodic echocardiography is recommended, considering the potential for tumor growth or recurrence after surgery[

16. Pericardial cyst Pericardial cysts are the most common pericardial tumors . They present as small, rounded, echolucent masses, most often adjacent to the right atrium. Usually, pericardial cysts are asymptomatic and have an excellent prognosis, but they can grow and compress the right cardiac chambers or the surrounding mediastinal structures[

17. Synopsis….Pericardial cyst Single Asymptomatic Surgery

18. Malignant tumors Malignant primary cardiac tumors are rare typically have rapid expansion, invading several cardiac structures (myocardium, cardiac chambers and pericardium).

19. Sarcoma: Sarcomas account for 95% of primary malignant cardiac tumors . They occur between the third and fifth decades of life, equally in both genders. Sarcomas are characteristically very aggressive, have a nonspecific clinical presentation and median survival is less than 1 year.

20. Rabdomyosarcoma Rhabdomyosarcomas represent the most frequent cardiac malignancy in infants and children. Rhabdomyosarcomas may occur in any cardiac chamber and in 60% of cases are multiple. Similarly to angiosarcomas, these tumors grow rapidly and are invasive, often extending to the pericardium before diagnosis[

21. Lymphoma Primary cardiac lymphomas are extremely rare, representing 5% of primary cardiac malignancies. They have increase prevalence in immunocompromised patients. They are defined as non-Hodgkin’s lymphoma located initially only in the heart or pericardium. Cardiac involvement in lymphomas is usually secondary and occurs by dissemination . Primary cardiac lymphomas involve more often the right side of the heart and usually associate with pericardial effusion. Prognosis is poor with less than 1 month survival without treatment and up to 5 years after radio- and chemotherapy

22. Mesothelioma Primary malignant pericardial mesothelioma is extremely rare and unlike pleural and peritoneal mesotheliomas has no definite correlation with exposure to asbestos. Echocardiographic findings are pericardial effusion, pericardial thickening or constrictive physiology. Invasion of other cardiac structures is rarely seen. poor prognosis with little benefit from radio and chemo-therapy.

23. Secondary cardiac tumours Extension to the heart is done by: haematogenous, lymphatic, direct or venous spread. Except for melanoma (very rare) that metastasis preferentially to the heart, cardiac metastasis are found in lung, breast, ovarian, kidney, leukemia , lymphoma, esophageal cancer patients in widespread dissemination stages. Echocardiography should be performed in all patients with a history of malignancy and cardiac symptoms. R enal cell carcinoma, hepatoma and uterine leiomyosarcoma (malignant) can extend to the right cavities through inferior vena cava. This is best visualised in the subcostal view. Cardiac metastases are predominantly located in the pericardium, with pericardial effusion being the most frequent finding. Chemotherapy and tumor resection may alleviate symptoms and prolong survival 

24. Diagnosis Echo’ CT MRI

25. Treatment Resection Follow up Work up