Lymphosarcoma

* We report a case of metastatic plasmacytoma to the myocardium and coronary vessels in a 57-year-old man with multiple myeloma. Originally, the patient had a large plasmacytoma in his left chest wall and lung. He received local radiation and chemotherapy. Subsequently, the patient presented with symptoms of congestive heart failure. He had no prior history of cardiac disease. The patient was treated medically and later died from respiratory failure. At autopsy, a metastatic plamacytoma was indentified within the myocardium and externally compressing the coronary arteries. The tumor infiltrated into the coronary sinus. It is difficult to speculate whether the patient's symptoms were due to cardiac involvement since the tumor burden in his chest was also considerable. To our knowledge, coronary vessel involvement with plasmacytoma has not been previously described.

(Arch Pathol Lab Med. 2000;124:910-912)

Metastatic tumors to the heart are seen in approximately 15% of autopsy patients with disseminated cancer. The most common malignancies that metastasize to the heart are lung and breast carcinoma, malignant melanoma, lymphoma, and leukemia. We report a case of metastatic plasmacytoma to the myocardium and coronary vessels in a patient with multiple myeloma.

REPORT OF A CASE

A 57-year-old man was seen in the emergency department with complaints of 2 weeks, duration of shortness of breath, orthopnea, and bilateral lower extremity edema. The patient had no prior heart disease and denied other cardiac symptoms. His medical history was contributory for obesity, hypertension, and multiple myeloma complicated by a large left lung and chest wall plasmacytoma. His initial multiple myeloma work-up 1 year previously showed a plasmacytoma of the left fourth rib, bone marrow biopsy with 30% plasma cells with atypical features, and a serum immunoglobulin G delta monoclonal spike. He received local radiation, melphalan and prednisone therapy, and 3 cycles of vincristine, Adriamycin (total dose, 228 mg), and dexamethasone before presenting with his current symptoms.

The patients blood pressure was 167/81 mm Hg, pulse rate, 96/min, and oxygen saturation, 96%. Physical examination revealed decreased breath sounds of the left lung field and crackles in the right lower lobe. A grade III/VI systolic ejection murmur and an S4 were auscultated. Bilateral 2+ pitting edema of the lower extremities was present. There was no jugular venous distention or Kussmaul sign. The electrocardiogram showed sinus tachycardia without evidence of ischemia. A large left upper lobe mass and blunting of the left costophrenic angle were seen on chest x-ray examination. Cardiac enzyme levels were normal. An echocardiogram was ordered for suspected chemotherapy-related cardiomyopathy. The study was technically limited due to the patients size and large plasmacytoma in his chest but did show mild aortic stenosis and mild tricuspid and mitral regurgitation. There was mild to moderate decreased left ventricular systolic function with lateral hypokinesis and mid inferior and septal akinesis. Additionally, a multigated acquisition scan showed a left ventricular ejection fraction of 60%io.

With the results of the cardiac studies, the probability of Adriamycin toxicity was low. The differential diagnosis for the patient's symptoms included diastolic dysfunction, transient ischemia, an intermittent arrhythmia, or hyopalbuminemia. No further workup was performed based on the status of his malignancy, and the patient was treated medically.

The patient received 1 more cycle of chemotherapy. He subsequently developed plasmacytomas of his thoracic spinal canal, right orbit, and frontal sinuses. Because of his unresponsiveness to treatment and further progression of disease, the patient chose to receive only palliative medical care. The patient progressively required oxygen therapy secondary to tumor burden in the thorax and malignant pleural effusions, respiratory failure developed, and the patient died.

AUTOPSY FINDINGS

At autopsy, there was an asymmetric chest wall with a mass protruding through the ribs and pectoral muscles on the left side. The left lung was collapsed and superiorly encased by tumor. There was a moderate pericardial effusion. A metastatic tumor was found on the posterior side of the heart wrapping around the great vessels and infiltrating into the adjacent myocardium encircling the right coronary artery sinus, and small arterial branches (Figure 1). The involved vessels were externally compressed with marked luminal narrowing. There was no atherosclerotic disease, and the remainder of the heart was unremarkable. The liver and spleen were grossly uninvolved with tumor.

Histologically, the tumor was cohesive, having large anaplastic plasmacytoid cells with cytoplasmic inclusions consistent with Russell bodies, a high mitotic rate with atypical mitoses, and scattered mature plasma cells. The tumor completely encased the right coronary artery with external compression of the vessel wall without invasion into the tunica media (Figure 2). The right atrium, ventricle, coronary sinus, and several nerve bundles were infiltrated by tumor (Figure 3). No amyloid was identified. There were no histologic changes in the heart to support Adriamycin toxicity. The sampled bone marrow had no evidence of plasmacytoma or multiple myeloma. The left lung was infiltrated with malignant plasma cells and had marked radiation changes. Also, a small serosal nodule on the stomach was found to be a plasmacytoma.

COMMENT

In general, metastatic tumors to the heart are more common than primary cardiac neoplasms. Tumor metastases to the heart are seen in approximately 15% of autopsy patients with disseminated cancer. They reach the heart by lymphatic and hematogenous spread or by direct invasion. The most common lesions originate from lung, breast, malignant melanoma, lymphoma, and leukemia.2 Extramedullary plasmacytoma of the heart is extremely rare. Keung et al3 reviewed the literature and found 9 cases involving the heart followed by a report of a case presenting as a cardiac emergency that required surgical intervention.

Metastatic involvement of the coronary vessels is reported even less often. The coronary vessels may be infiltrated or compressed by tumor, or they may have carcinomatous emboli lodged in the lumina. Fishberg4 reported a case of lymphoma surrounding the left circumflex artery in 1930. A woman with metastatic fibrosarcoma occluding a coronary artery was reported by Frolich et al.5 Javier et alb also described a patient with Hodgkin disease in review of the literature. To our knowledge, coronary vessel involvement with plasmacytoma has not been previously described.

Metastatic disease of the heart can present as angina, congestive heart failure, pericarditis, or with an arrhythmia? Retrospectively, the incidence of cardiac symptoms presumed related to the cardiac metastases identified at autopsy ranges from 20%8 to 43%; however, only half of these are ascribed clinically premortem as to being the cause of the symptoms.1,9 It is often difficult at autopsy to determine which cardiac symptoms were related to malignant cardiac infiltrates.10-12 We can only speculate that the patient's cardiac symptoms were a direct result of his large tumor burden. The patient had evidence of extrinsic cardiac compression, which by itself may have led to a picture typical for constrictive physiology. We must also consider the possibility of intermittent coronary ischemia caused by either external coronary artery compression or direct intraluminal vessel invasion by tumor. All 3 of these possibilities were present at autopsy. Cardiac catheterization may have helped to separate the exact etiology, but the patients dismal short-term prognosis did not warrant performance of the procedure.

Therefore, in patients with an underlying malignancy who later present with new-onset heart failure symptoms, it is important to consider cardiac metastases in the differential diagnosis with close examination of the heart at autopsy for tumor involvement or therapy-related changes.

References

1. Burke AP, Renu V. Tumors of the Heart and Great Vessels. Washington, DC: Armed Forces Institute of Pathology; 1996:195-197. Atlas of Tumor Pathology; 3rd series, fascicle 16.

2. Hanfling S. Metastatic cancer to the heart: review of the literature and report of 127 cases. Circulation. 1960;22:474-483.

3. Keung YK, Lau S, Gill P. Extramedullary plasmacytoma of the heart presenting as a cardiac emergency: review of literature. Am] Clin Oncol. 1994;17: 427-429.

4. Fishberg A. Auricular fibrillation and flutter in metastatic growths of the right auricle. Am J Med Sci. 1930;180:629-629.

5. Frolich E, Schnider B, Leonard J. Antemortem diagnosis of metastatic sarcoma to the heart: a case report. Heart]. 1959:57:623-629.

6. Javier BV, Yount WJ, Crosby DJ, Hall TC. Cardiac metastases in lymphoma and leukemia. Dis Chest. 1967;52:481-484.

7. Franciosa J, Lawrinson W. Coronary artery occlusion due to neoplasm: a rare cause of acute myocardial infarction. Arch Intern Med. 1971;128:797-801. 8. Chan HS, Sonley MJ, Moes CA, Daneman A, Smith CR, Martin DJ. Primary

and secondary tumors of childhood involving the heart, pericardium, and great vessels: a report of 75 cases and review of the literature. Cancer. 1985;56:825836.

9. Adenle AD, Edwards JE. Clinical and pathologic features of metastatic neoplasms of the pericardium. Chest. 1982;81:166-169.

10. Glancy DL, Roberts WC. The heart in malignant melanoma: a study of 70 autopsy cases. Am J Cardiol. 1968;21:555-571.

11. Roberts WC, Bodey GP, Wertlake PT. The heart in acute leukemia: a study of 420 autopsy cases. Am j Cardiol. 1968;21:388-412.

12. Roberts WC, Glancy DL, DeVita VT. Heart in malignant lymphoma (Hodgkin's disease, lymphosarcoma, reticulum cell sarcoma, and mycosis fungoides): a study of 196 autopsy cases. Am I Cardiol. 1968;22:85-107.

Accepted for publication November 17, 1999.

From the Department of Pathology (Drs Champeaux, Blaser, and Myers) and the Department of Internal Medicine, Cardiology Services (Dr Schachter), Madigan Army Medical Center, Tacoma, Wash.

The views expressed herein are those of the authors and do not necessarily represent the official position of the Department of the Army or the Department of Defense.

Reprints: Anne L. Champeaux, MD, Department of Pathology, Madigan Army Medical Center, Tacoma, WA 98431.

Copyright College of American Pathologists Jun 2000
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