Cardiac Tumor

1. Cardiac Tumor Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery

2. 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

3. Primary Cardiac Tumors • Introduction • Primary cardiac tumors are uncommon clinical entities with anincidence of 0.0017% to 0.03% • The majority of thesetumors are benign atrial myxomas, successfullymanaged by surgical excision • Malignant cardiac tumors continue to present a difficult therapeutic challenge,and surgical resectionis often necessary to alleviate the severe symptoms associatedwith these tumors, but is, nevertheless, associated with poorlong-term prognosis • Because of the rarity of primary cardiacmalignancies, therapeutic concepts and methods of surgical resectionhave not been standardized.

4. 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

5. 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)

6. 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

7. 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.

8. 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

9. 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.

10. 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

11. 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.

12. 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

13. 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

14. 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.

15. 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

16. 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

17. 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.

18. 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.

19. 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

20. Rhabdomyoma

21. 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

22. 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

23. Cardiac Fibroma (Gross)

24. Cardiac Fibroma (Microscopy)

25. Malignant Cardiac Tumors • Introduction • Malignant tumors continue to present a therapeutic challengeas incomplete resections universally result in rapid local tumorrecurrence • Resectionof left atrial tumors include radical resections using leftatrial approaches through the interatrial groove or transseptallythrough the right atrium. • For left ventricular tumors,surgical resection can be accomplished through a transaorticvalve approach, a ventriculotomy, or a transmitral valve approach. • Cardiac autotransplantation is a technique witha novel application for resection of malignant cardiac tumors • Orthotopic heart transplantation and combined heart-lungtransplantation have been previously described

26. Angiosarcoma

27. 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 .

28. 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.

29. Ventricular Outpouchings • Introduction • Congenital ventricular outpouchings include congenital ventriculardiverticulum (CVD) and aneurysm (CVA) • Although variouscharacteristics have been proposed to differentiate the twoentities, definition remains incomplete. • In addition, theirembryologic origin, clinical profile, natural course, and treatmentare unclear since these malformations are rare

30. Ventricular Outpouchings • Definition • CVD was characterized by synchronous systolic contractility, which was correlated to the presence of muscular fiberswithin the diverticular wall on histologic examination. • When apical, CVD was a fingerlikecontractile pouch with narrow connection to the ventricle ands a part of the Cantrell syndrome • When nonapical, CVD was a large contractile pouch with wideconnection to the ventricle and was never associated with intracardiacor extracardiac abnormalities. • In contrast, CVA was a large akinetic or dyskinetic pouch withwide connection to the ventricle and endocardial or transmuralfibrosis on histologic examination. • CVA was never associated with intracardiac or extracardiac defects.

31. Ventricular Outpouchings • Etiology • Apical CVD is a part of the Cantrell syndrome and has a common defect in embryologic development with midlinethoracoabdominal formation • The development of nonapical CVD and CVA has been attributed toa focal defect of the muscular ventricular wall due to an intrinsicabnormality in embryogenesis. • CVA has also been suspectedto be acquired in the prenatal period, potentially as a resultof a viral infection or coronary lesions including stenosis,hypoplasia, and localized intimal proliferation.

32. Ventricular Outpouchings • Prognosis and outcome • The prognosis for apical CVD depended on associated intracardiacmalformations and was generally good after repair. • The outcomefor nonapical CVD was also excellent, with even a total regression in some cases • CVA hada poor prognosis with frequent fatal cardiovascular complicationsin the neonatal period. Usually diedof heart failure within a few days after birth. Poor prognostic factorssuch as size and progression ofthe CVA and signs of cardiac failure.

33. Ventricular Outpouchings • Treatment • When symptomaticor when associated with other cardiac abnormalities, surgicaltreatment is usually recommended. • Controversy concerns whetheran asymptomatic and isolated CVD should be treated surgically. • CVD can potentially give rise to embolism, arrhythmias, cardiacfailure, and rupture • A different approach be considered for asymptomatic CVAwith severe endocardial fibrosis or transmural fibrosis becauseparadoxical contractions cause dilatation and expose the aneurysmto the risk of rupture.