|Year : 2010 | Volume
| Issue : 3 | Page : 347-349
Primary right atrial sarcoma
Virendra Bhandari1, Pramod Sakhi1, Kavita Munjal1, Amit V Varma1, Rakesh Shishodiya2, Vishal K Jain2
1 Department of Radiotherapy, Sri Aurobindo Institute of Medical Sciences, Indore, India
2 Department of Surgery, Sri Aurobindo Institute of Medical Sciences, Indore, India
|Date of Web Publication||29-Nov-2010|
401, Samyak Towers, 16/3, Old Palasia, Indore - 452 001, Madhya Pradesh
Source of Support: None, Conflict of Interest: None
Primary malignant tumors of heart are rare with unfavorable prognosis and are mostly diagnosed on autopsy. They have varied presentation and are difficult to diagnose. Most of the cases are benign and others are sarcomas. Here we present a case of primary cardiac sarcoma which had varied presentation and was undiagnosed due to non specific symptoms. Diagnosis of atrial tumor was confirmed on histopathological examination of tissue obtained after thoracotomy. Being inoperable, the patient was given palliative chemotherapy with which we could provide her a better quality of life.
Keywords: Cardiac sarcoma, palliation, primary right atrial tumor, varied presentation
|How to cite this article:|
Bhandari V, Sakhi P, Munjal K, Varma AV, Shishodiya R, Jain VK. Primary right atrial sarcoma. J Can Res Ther 2010;6:347-9
| > Introduction|| |
Cardiac sarcomas are uncommon entities. Only about 300 cases have been reported till date. Seventy percent of cardiac masses are benign presenting as myxomas and rest present as sarcomas. Clinical presentation also varies.The diagnosis of sarcoma cannot be made preoperatively because of nonspecific signs and symptoms. The sites involved, in decreasing order of frequency, are the right atrium, left atrium, right ventricle and left ventricle. 
| > Case Report|| |
A 21year old female came with dyspnoea on exertion, high grade fever, generalized weakness and chest pain of seven days duration. She was breathless, normotensive with a pulse rate of 76/min. There was no lymphadenopathy and jugular venous pressure was normal. Minimal generalized edema was present. Heart sounds were muffled. A clinical diagnosis of pericardial effusion was made. In past six months, patient has been treated as tubercular pericardial effusion and nephrotic syndrome at different centers, without any relief.
Routine blood investigations were normal. There was massive cardiomegaly. Echocardiogram showed pericardial effusion with temponade and fibrous bands in pericardial sac. The CT scan revealed gross pericardial effusion with multiple small mediastinal lymph nodes.
Diagnostic midline sternotomy was performed for lymph node biopsy and a mass was seen on right side in heart muscles invading the pericardium thereby making the differentiation of tissue difficult. Multiple biopsies were taken from the mass. A diagnosis of high grade spindle cell sarcoma of right atrium was made after histopathological examination [Figure 1]. Immunohistochemistry was not performed. A repeat CT scan revealed large mass in the right atrium [Figure 2]. Patient was advised to take palliative chemotherapy which the family members denied.
|Figure 1: Microphotograph showing malignant spindle cells with brisk mitosis. H & E ×400)|
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|Figure 2: Contrast enhanced scan of thorax showing mass in right atrium indenting the atrial wal|
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The patient presented after two months with acute shortness of breath and orthopnoea and raised Jugular Venous Pressure. The routine investigations were normal, so pericardiocentesis was done and chemotherapy with cyclophosphamide, vincristine and actinomycin was given as patient could not afford expensive curative chemotherapy with ifosphamode and adriamycin. Within four days, the patient responded and was able to do her routine work. She received four cycles of chemotherapy in four months. During this time, the patient remained comfortable and could walk up to one kilometer without rest. Her echocardiography still showed pericardial effusion and mass in the right atrium [Figure 3]. She was lost to follow up and reported back after four months with severe respiratory distress and expired. She survived for eleven months after diagnosis with maximum symptom free duration.
| > Discussion|| |
This patient had a right atrial sarcoma with associated pericardial effusion. The patient presented with atypical signs and symptoms which included peripheral edema, dyspnoea, high grade fever, weakness and chest pain based on which she was treated as pericardial tuberculosis and nephrotic syndrome in the past. The right atrial mass was missed on sonography and echocardiography and CT scan initially.
Primary cardiac neoplasms are rare with an estimated frequency of 0.0017- 0.33%. , A review showed incidence of 0.05% as primary cardiac lesions and 1.23% as secondary tumors. The mean age at which primary and secondary sarcomas involve the heart is 55 years and 35 years, respectively. ,, Cardiac sarcomas are extremely rare in pediatric age and are reportedly more common in males than in females. ,
Seventy-five percentage of cardiac masses are benign and 25% are malignant, of which 95% are sarcomas and 5% are primary lymphomas. , Spindle cell sarcomas account for approximately 5% of all cardiac tumors. An association between cardiac sarcoma and asbestos exposure is seen. There is a predilection for the atria and pericardial surfaces in cardiac sarcomas.  Similar right atrial involvement along with pericardial infiltration is seen in our case.
Clinical presentation depends on the size of tumor and its anatomic location. Tumor type, growth rate, friability and invasiveness are also important factors that determine clinical features.  Large tumors may be relatively silent, whereas small tumors in critical location may give rise to devastating consequences. Left atrial tumors may cause systemic embolization leading to myocardial infarction or may interfere with mitral valve to cause stenosis. Dyspnoea is reportedly the most common presentation and in left atrial lesions it is attributed to venous obstruction. ,,
Intramural left ventricular tumors may remain asymptomatic. Right atrial and ventricular tumors may result in heart failure from atroventricular or pulmonary outflow obstruction resulting in peripheral edema, hepatomegaly, ascitis, and shortness of breath, syncope and sometimes sudden death. , Our patient also presented with shortness of breath and peripheral edema.
The prognosis for cardiac sarcomas remains dismal because of (i) advanced tumor stage at presentation, (ii) nonspecific symptomatology, (iii) insufficient awareness, (iv) delayed diagnosis and/or misdiagnosis. Thus, a vicious cycle is created.  Consequently, there exists no consensus on therapeutic modalities for cardiac sarcomas.
Kim et al. reported a case of a primary cardiac sarcoma with myxoid change.  On operating, the mass was found to extend into the posterior left atrial wall, left pulmonary vein, and mitral valve. Wide resection of the left atrium, mitral valve replacement and left pneumonectomy was done. The histological diagnosis was undifferentiated primary cardiac sarcoma. The patient received postoperative chemotherapy and expired 11 months after surgery due to local recurrence. The resistance of cardiac sarcomas to radiotherapy and chemotherapy complicates the management of these lesions.  The limited number of cases prevents drawing solid conclusions about the effectiveness of therapy.
In a series of 14 cases reported by Frank Mayer et al. 11 patients received palliative chemotherapy. Eight patients attained response to chemotherapy and two had disease stabilization for 6 to 12 months concluding that in advanced disease chemotherapy gives promising response. 
In this case, we saw definite partial response with combination chemotherapy. There was reduction in size of the tumor and pericardial infiltration. A combination of newer chemotherapeutic drugs like adriamycin, ifosphamide may definitely help in increasing the response rate as well as overall survival.
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[Figure 1], [Figure 2], [Figure 3]