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Journal of Clinical Oncology, Vol 25, No 10 (April 1), 2007: pp. 1277-1279 © 2007 American Society of Clinical Oncology. DOI: 10.1200/JCO.2006.09.9077
Left Atrial Sarcoma Mimicking Obstructive Pulmonary DiseaseDivision of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
Division of Hematology-Oncology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
Division of Cardiovascular Surgery, Department of Surgery, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
Division of Cardiology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
Department of Pathology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
Division of Hematology-Oncology, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan A 54-year-old man presented to our hospital with progressive nonproductive cough for 1 month. He also complained of exertional dyspnea, chest tightness, and wheezing for 2 weeks. The symptoms were characterized by night attacks when he usually awoke to cough and breathe. On physical examination, an expiratory wheezing sound was heard. He did not have heart murmur, nor did he have pitting edema on his legs. Accordingly, he was suspected to have asthma. The spirometry confirmed an obstructive type pulmonary disorder. However, the bronchodilator test was negative. Surprisingly, his chest radiograph showed increased infiltrations over the left upper lobe with lung volume reduction (Fig 1). Because he was not febrile and had a negative bronchodilator test, a bronchogenic carcinoma with partial obstruction of the left upper lobe was therefore taken into differential diagnosis. More surprisingly, chest computed tomography scans revealed a huge tumor mass in left atrium (Figs 2A and 2B; arrows) with invasion into left superior and inferior pulmonary veins (Figs 2A and 2B; arrow heads). Because of the invasive nature of the tumor, a malignancy was favored. Although the computed tomography scans failed to demonstrate a main mass in the lung fields, the higher incidence of primary lung cancer than that of primary cardiac cancer and occasional reports of intravenous extension of lung cancer to pulmonary veins and left atrium1-3 made the primary pathology of lung cancer or cardiac cancer in debate. Bronchoscopy revealed stenotic bronchial lumens in the left upper lobe, lining with irregular, fragile, hyperemic, and edematous mucosa (Fig 3A), compared with the normal bronchus in the right lung (Fig 3B). Morphologically, the findings of bronchoscopy somewhat mimicked the submucosal type of lung cancer. However, the cytological and pathological examinations obtained from bronchoscopy showed negative for malignancy throughout the submucosal layer.
Despite the existence of a huge left atrial mass and pulmonary vein obstruction, his left ventricular function was normal on echocardiography. His ECG was unremarkable. A thorough evaluation, including positron emission tomography, helped to exclude the possibility of distant metastases. However, the extensive tumor invasion of the pulmonary veins made a curative surgery impossible. Still, he underwent palliative surgical resection of the cardiac tumor to obtain tissue diagnosis and to prevent the possible tumor obstruction of mitral valve, which may result in sudden death. The surgical pathology proved a sarcoma from left atrium. The pathological examinations (Fig 4) demonstrated that the cardiac tumor was large in size and had bizarre-shaped neoplastic cells exhibiting eosinophilic and indistinct-bordered cytoplasm (hematoxylin and eosin stain; original magnification x400). The immunohistochemical studies demonstrated positive reaction with vimentin (Fig 4, inset right) and negative reaction with CD34 (Fig 4, inset left; original magnification x200). Because the morphological and immunohistochemical studies failed to subclassify this tumor into any specific type of sarcoma, a final diagnosis of undifferentiated sarcoma was made. As expected, the surgery did not significantly improve his symptoms. After recovery from the surgery, he was treated with mesna, doxorubicin, ifosfamide, and dacarbazine (MAID) chemotherapy for two cycles followed by radiotherapy. Unfortunately, his cardiac sarcoma responded poorly to any anticancer treatments. Local recurrence and multiple brain metastases occurred, and he died 5 months after diagnosis.
The differential diagnosis of cardiac tumor in our patient included atrial myxoma, atrial sarcoma, metastasis from other primary malignancy, particularly from lung cancer as mentioned, and other less frequent cardiac neoplasms. Myxoma is by far the most common primary cardiac tumor in adults, comprising 50% of the tumors. Ninety percent of these tumors are left atrial.4 Because there are no pathognomonic symptoms and signs for atrial myxoma, the differential diagnosis cannot be made only on clinical grounds. However, the relatively benign nature of this cardiac tumor made it less likely for our patient to have atrial myxoma. The image studies demonstrated the invasive nature of cardiac tumor in our patient. Primary cardiac sarcomas, the second most common primary cardiac tumor, are often asymptomatic until advanced, and even then produce nonspecific symptoms and mimic other pathology. The clinical presentations vary depending on the sites of involvement. Several mechanisms are proposed for the manifestations of cardiac sarcoma, including acute heart failure, blood flow obstruction, pericardial effusion with tamponade, arrhythmia caused by local invasion, and embolic phenomena.5 Our patient presented with airway obstruction, which was confirmed by lung function test and was visualized on bronchoscopic examination. The airway edema and stenosis might be caused by tumor obstruction of pulmonary veins. Moreover, the observation that a single bronchoscopic biopsy attempt resulted in severe bleeding implied marked congestion of the bronchial vessels. To the best of our knowledge, this is the first reported case of cardiac sarcoma that presented as an obstructive lung disease. Primary cardiac sarcomas are associated with delayed diagnosis and high incidence of hematogenous metastases, and therefore, carry a dismal prognosis. The mean survival is about 9.6 to 16.5 months.6,7 Currently, only early diagnosis with complete surgical resection provides the potential for long-term survival. Clinicians should be familiar with the presentations of cardiac sarcomas. With a high index of clinical suspicion and the availability of modern imaging technology, earlier diagnosis is always possible. AUTHORS' DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The authors indicated no potential conflicts of interest.
REFERENCES 1. Mehan VK, Deshpande J, Dalvi BV, et al: Direct extension of bronchogenic carcinoma through pulmonary veins into the left atrium mimicking left atrial myxoma. Chest 101:1722-1723, 1992[Medline] 2. Desai MY, Mankad S: Extension of bronchogenic carcinoma through pulmonary vein into the left atrium detected by echocardiography. Echocardiography 21:189-191, 2004[CrossRef][Medline] 3. Kodama K, Doi O, Tatsuta M: Unusual extension of lung cancer into the left atrium via the pulmonary vein. Int Surg 75:22-26, 1990[Medline] 4. Shapiro LM: Cardiac tumours: Diagnosis and management. Heart 85:218-222, 2001 5. Shanmugam G: Primary cardiac sarcoma. Eur J Cardiothorac Surg 29:925-932, 2006 6. Donsbeck AV, Ranchere D, Coindre JM, et al: Primary cardiac sarcomas: An immunohistochemical and grading study with long-term follow-up of 24 cases. Histopathology 34:295-304, 1999[CrossRef][Medline] 7. Bakaeen FG, Reardon MJ, Coselli JS, et al: Surgical outcome in 85 patients with primary cardiac tumors. Am J Surg 186:641-647, 2003[CrossRef][Medline]
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Copyright © 2007 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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