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Journal of Clinical Oncology, Vol 25, No 4 (February 1), 2007: pp. 449-451 © 2007 American Society of Clinical Oncology. DOI: 10.1200/JCO.2006.09.2346
Cardiac Primitive Neuroectodermal Tumor Presenting As Acute Coronary SyndromeNizams Institute of Medical Sciences, Hyderabad, India A 40-year-old man, nonsmoker, nondiabetic, normotensive was admitted with the complaint of left side chest pain of a day's duration. The pain was gripping in nature, radiated to left arm, and it was associated with nausea and vomiting. On examination pulse, blood pressure, and temperature were normal. Systemic review was unremarkable. An ECG showed ST-T changes suggestive of acute anterior wall non-ST segment elevation myocardial infarction (Fig 1). A two-dimensional echocardiogram showed a mass obliterating the apex of the both ventricles with good biventricular function. There were no significant valvular abnormalities, clot, or pericardial effusion. Computed tomography angiogram of the heart (Fig 2) revealed a large, well-defined exophytic mass measuring 7 cm (width) x 9 cm (cranio-caudal) x 5.6 cm (antero-posterior) anterior to the heart, involving the interventricular septum near apex and parts of the adjacent ventricular myocardium, without any evidence of spread beyond the pericardium. The distal left anterior descending artery was encased by the tumor causing more than 90% narrowing. Magnetic resonance imaging of the heart confirmed the presence of mass, which was showing heterogeneous intensity with a few cystic areas. A coronary angiogram confirmed the tumor causing 99% obstruction of distal left anterior descending artery by deviation and compression. At thoracotomy, the mass was found to be inoperable hence a biopsy was done. Histopathologic examination revealed a tumor with islands and nests of relatively monomorphous small blue cells with round hyperchromatic nuclei and scant cytoplasm, diffusely infiltrating the collagenous tissue (Figs 3 and 4; magnification of x40 and x100, respectively). Rosettes were not seen. Immunohistochemistry showed strong cytoplasmic membrane positivity for CD-99 in the tumor cells (Fig 5; magnification x200). Immunostaining for chromogranin, desmin, leukocyte common antigen, and cytokeratin were all negative. Hence, a diagnosis of cardiac primitive neuroectodermal tumor/Ewing's sarcoma was made. Staging work-up was negative for metastasis. The patient was started on chemotherapy with ifosphamide and etoposide alternating with vincristine, doxorubicin, and cyclophosphamide (IE/VAC). Repeat evaluation with a two-dimensional echocardiogram after two cycles of chemotherapy showed a 50% regression in the size of the mass with good symptom relief and reversal of all the ECG changes.
Primary cardiac tumors are infrequent and the majority benign. The incidence varies from 0.001% to 0.28% in autopsy series.1 Sarcomas are the most common primary malignant cardiac tumors followed by lymphoma and mesothelioma.2 Primitive neuroectodermal tumors of the heart are extremely rare. To our knowledge only two cases have been reported earlier of which one was in the right ventricle and other in the right atrium.3,4 Symptoms are generally nonspecific and are due to dysfunction of the myocardium, pericardium, or valves.2 These include congestive heart failure, myocardial ischemia, thromboembolism, arrhythmias, or invasion of adjacent mediastinal structures.5 While the previously reported cases had a subacute history with exertional dyspnea, fatigue, and weight loss, our patient had an acute presentation with symptoms of acute myocardial ischemia and ECG features of non-ST segment elevation myocardial infarction. Transthoracic echocardiography is the investigation of choice for initial evaluation. Computed tomography and magnetic resonance imaging help to further characterize the tumor and to assess the extent of extra cardiac spread.6,7 Light microscopic examination of the tumor shows diffuse sheets of round cells separated by strands of fibrous tissue. Homer-Wright pseudorosettes may or may not be present.3 The round cells show strong membrane positivity for CD-99 with negative staining for cytoketatin, desmin, and leukocyte common antigen. Primitive neuroectodermal tumor shows positivity for neural markers like neuron specific enolase, synaptophysin, and chromogranin. Classical cytogenetic abnormalities include reciprocal translocations t(11; 22) or t(21; 22).3 Malignant cardiac tumors portend a poor prognosis.8 Due to the invasive and infiltrative nature of malignant tumors surgery is rarely curative. Chemotherapy and radiotherapy may be useful to palliate symptoms. AUTHORS' DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The authors indicated no potential conflicts of interest.
REFERENCES 1. Abraham JM: Neoplasms metastatic to the heart: Review of 3314 consecutive autopsies. Am J Cardiovasc Pathol 3:195, 1990[Medline] 2. Perchinsnky MJ, Lichtenstein SV, Tyers GF: Primary cardiac tumors: Forty years' experience with 71 patients. Cancer 79:1809-1815, 1997[CrossRef][Medline] 3. Charney DA, Charney JM, Ghali VS, et al: Primitive neuroectodermal tumor of the myocardium: A case report, review of the literature, immunohistochemical and ultrastructural study. Hum Pathol 27:1365-1369, 1996[CrossRef][Medline] 4. Besirli K, Arslan C, Tuzun H, et al: The primitive neuroectodermal tumor of the heart. Eur J Cardiothorac Surg 18:619-621, 2000 5. Roberts WC: Primary and secondary neoplasms of the heart. Am J Cardiol 80:671-682, 1997[CrossRef][Medline] 6. DePace NL, Soulen RL, Kotler MN, et al: Two dimensional echocardiographic detection of intraatrial masses. Am J Cardiol 48:954-960, 1981[CrossRef][Medline] 7. Araoz PA, Eklund HE, Welch TJ, et al: CT and MR imaging of primary cardiac malignacies. Radiographics 19:1421-1434, 1999 8. Piazza N, Chughtai T, Toledano K, et al: Primary cardiac tumors: Eighteen years of surgical experience on 21 patients. Can J Cardiol 20:1443-1448, 2004[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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