CHAPTER VIII - Primary and Secondary Cardiac Tumours - Marian GASPAR 8. 1. Introduction - History Although cardiac tumours have been described on anatomical parts by doctors since the 17th century, their antemortem diagnosis was possible much later with the introduction of angiographic and ultrasound study after 1950. For this reason and due to the emergence of ECC, the first cardiac tumour resection surgery took place in 1955. Classification of cardiac tumours Cardiac tumours are primary or secondary as the initially affected structure is the heart or organs in the vicinity, from which cardiac structures are invaded. After the invasion character they are divided into benign and malignant tumours (Table I). Table I. Classification of cardiac tumours Benign cardiac tumours Malignant cardiac tumours - Myxoma - Lipoma - Rhabdomyoma - Fibroma - Angioma - Teratoma - Pericardial cyst - Angiosarcoma - Rhabdomyosarcoma - Mesothelioma - Fibrosarcoma - Leiomyosarcoma - Liposarcoma Primary cardiac tumours Incidence of primary pericardial and heart tumours is from 0.001 to 0.28%. Fetal echocardiographic studies performed multicentrically in over 14000 cases detected primitive cardiac tumours in 17 faeces (0.14%). Diagnosis was made between 21 st to 38 th week of pregnancy. Among them the most common ones are cardiac myxomas, representing 50% of benign primary tumours. Table II. Signs and symptoms of heart tumours Location Clinical manifestations PERICARDIAL TUMOURS - pain - pericardial fluid - enlargement of the Rx heart image - tamponade - constriction MYOCARDIAL TUMOURS - rhythm and driving disorders - ECG changes - increasing Rx cardiac shadow - congestive heart failure - angina pectoris INTRACAVITARY TUMOURS - obstruation of valvular orifices - systemic embolisms 1
8. 2. Clinical signs of tumours. Cardiac tumours and especially myxomas can produce a wide range of local and systemic manifestations like fever, fatigue, purple rash, arthralgia, peripheral and cerebral embolisms, ocular manifestations, behavioural problems, pericardial effusion and compressions on various structures. Tumour signs and symptoms are due to their location rather than their histopathological form (Table II). All these deceptive signs delay the diagnosis. 8. 3. Clinical and paraclinical diagnosis. Clinical signs of tumours are often misleading, suggesting other cardiac or systemic diseases, resulting in delayed diagnosis and advancing tumour, sometimes to complications or inoperability. Clinical suspicion caused by blows, pericardial effusion without other etiology, is the most important time in orienting the diagnosis, which is confirmed by investigations of great precision like transesophagian echocardiography, CT scan, MRI, angiography. Sometimes a routine x-ray can bring evidence of mediastinum widening, pericardial effusion, pleural, calcification in the cardiac shadow. 8. 4. BENIGN CARDIAC TUMOURS Benign cardiac tumours are: myxoma, lipoma, rhabdomyoma, fibroma, angioma, teratoma and pericardial cyst. 8. 4. 1. Cardiac myxomas Cardiac myxomas, otherwise rare, are the most common cardiac tumours. Their incidence in cardiac surgery is between 0.0013-0.005% (D. Cooley -1984). Usually located in the left atrium with adherence to the interatrial septum, they may be seen in the right or bilateral atrium very rarely, attached to mitral valve, the tricuspid valve, or even more rarely 5% in ventricles. They occur more frequently in adults, with prevalence in women but can also occur in children and family. Symptomatic spectrum is very wide; cardiac myxomas can feign a number of cardiovascular diseases, infectious, collagen diseases. Fig 1. Echocardiography outlining well circumscribed tumour in LA with prolabation in mitral orifice. The diagnosis most often made in these situations is mitral stenosis. The diagnosis is very often suspected by history, clinical examination, ECG, Rx-chest (5% in large series). Once suspected this possibly fatal disease, the diagnosis is easy, confirmed by echocardiographic transthoracic transesophagian examination, or more sophisticated CT-scan and MRI (Figure 1). It takes several months from the onset of symptoms until the diagnosis. 2
Among the rare diseases that atrial myxomas have feigned there are Ortner Syndrome, Ehrmann-Sneddon Syndrome (livedo racemosa, associated with cerebrovascular lesions), coronary lesions (coronary steal syndrome), rheumatic disease in children, peripheral brain thromboembolic disease or sudden death. Most feared complications of cardiac myxomas are embolisms, especially the brain and sudden death in mitral orifice obstruation. Treatment is surgical, immediately after diagnosis and consists of tumour resection and eventually restoring the structure of insertion (interatrial septum, mitral tricuspidal valve). The prognosis of patients in surgery is excellent. First resection was performed in 1954 in Sweden by Clearence Crafoord, the patient is still alive. Recurrence of cardiac myxoma is explained by inadequate resection, multiple centres of seeding, family type, metastases. In conclusion, cardiac myxoma is a benign tumour, misleading in the clinical forms that it takes, potentially morbid and even lethal, which must justify doctors vigilance in early diagnosis. Once diagnosed, the only rational and immediate treatment is the surgical one. Rhabdomyoma. It is the most common tumour seen in children. It can be found in LV, RV and it is usually multiple. Its signs are cardiomegaly with ventricular dysfunction, feigning valvular diseases, like mitral, pulmonary stenosis. Intracavitary obstruction can lead to death. Fibroma. It is a benign tumour of connective tissue that occurs most frequently in children. It develops in the ventricular myocardium, a circumscribed firm formation, 4-10 cm in size. It causes driving disorders, arrhythmias; it alters myocardial contractility and can cause an obstacle in the hemodynamics of that cavity. Diagnosis is based on echocardiography, CT, MRI during life or post-mortem at autopsy. Surgical resection under ECC is possible in most cases. Lipoma occurs at any age, affecting LV, LA, interatrial septum, reaching dimensions of several inches, polypoid or sessile in shape. It is well-defined, encapsulated and causes driving disorders or obstructive phenomena. Angioma. It represents a rare vascular tumour, occurring intramurally with polypoid, sessile aspect, like red subendocardial nodules, which can break and cause cardiac tamponade. 8. 4. 2. SURGICAL TREATMENT OF BENIGN TUMOURS Once made the diagnosis of benign cardiac tumour, the treatment is immediate surgery and consists of complete resection of the tumour under ECC, with the restoration of the insertion structure, interatrial septum, tricuspidal or mitral valve (Fig 2.). The excision surgery of cardiac myxomas is simple, with low morbid potential and excellent immediate and longterm prognosis. However, recurrent tumour is possible, either due to incomplete resection or incipient outbreaks that develop after surgery. If it is epicardially located, resection is done without using ECC. 3
Fig 2. Left atrial myxoma, intraoperative image and the removed piece 4 / 6 cm (Clinic of Cardiac Surgery-M. Gaspar & H. Antretter, Innsbruck, Austria, 2002) 8. 5. MALIGNANT CARDIAC TUMOURS The malignant cardiac tumours are: angiosarcoma, rhabdomyosarcoma, mesothelioma, fibrosarcoma, leiomyosarcoma, liposarcoma and osteosarcoma. Malignant tumours are ¼ of cardiac tumours and the most common ones are sarcomas. Sarcomas result from proliferation of mesenchymal tissue, showing a variety of morphological types, angiosarcomas, fibrosarcomas, rhabdomyosarcomas, osteomas. They are common at any age but mostly in the third, fourth decade of life, located in the RA, LA, RV and LV. They are very aggressive tumours that lead to patient s death within weeks to two years after the onset of clinical manifestations. Cardiac signs are determined by location and extension of tumour, with pleural effusion, nonspecific chest pain, fatigue, weight loss, loss of appetite, various arrhythmias and sudden death. 8. 5. 1. Angiosarcomas They have a particular tropism for the right atrium, where they develop in a diffuse, infiltrative way, affecting structures at this level. Diagnosis is difficult to make in the early stages due to nonspecific symptoms, fatigue, chest pain, night sweats, weight loss, loss of appetite, pericardial effusion, patients being often under suspicion of tuberculosis. The first step to diagnosis is clinical suspicion followed by laboratory investigations MRI, CT, echocardiography, angiography, pericardoscopy. Tumour spread to surrounding structures and at distance is quite fast (Fig. 3). 4
Fig 3. Angiosarcoma of the right atrium. Patient BM, 45, was operated in Cardiac Surgery Clinic in Vienna, but died six months later by pulmonary metastases (Prof. Dr. E. Wolner, Dr. M. Gaspar, Vienna, 1996). The prognosis is according to the histological type of the tumour, those presenting necrosis, acellular elements, being the most aggressive. Once diagnosed, treatment is as urgent and complex as possible, surgery, radiation and chemotherapy. Rhabdomyosarcomas. They derive from chequered muscle structure and can be diffuse by infiltrating the myocardium or rarely polypoids, extended to cardiac cavities and similar to myxomas. Lymphomas. They are either located cardiacally or as a result of systemic lymphomas. They may be nodular or diffuse, even intracavitary, leading to blockages. However, they respond well to chemotherapy and radiation. 8. 5. 2. SURGICAL TREATMENT OF MALIGNANT CARDIAC TUMOURS There is no consensus regarding therapy in case of malignant cardiac tumours. Some authors are for surgical resection, as radical and early as possible. Others say that surgical intervention would only have the diagnostic role of biopsy. For most radical resection under the ECC, the autotransplantation technique may be used, that is the heart is removed from the pericardial cavity, the tumour is resected, structures recover and the heart is reimplanted. Orthotopic heart transplantation in malignant tumours is not unanimously consensuous either, did not give the expected results and is contraindicated by some authors. Therefore, prognosis of these tumours remains dismal. 5