Jpn J Clin Oncol 2002;32(11)480–482
Giant Metastatic Myxoid Liposarcoma Causing Cardiac Tamponade: a Case Report Shelin P. T. Wong, Calvin S. H. Ng, Song Wan, Tak-Wai Lee, Innes Y. P. Wan, Anthony P. C. Yim and Ahmed A. Arifi Division of Cardiothoracic Surgery, The Chinese University of Hong Kong, Prince of Wales Hospital, Sha Tin, NT, Hong Kong Received May 7, 2002; accepted August 23, 2002
We report a case of a rapidly progressing isolated giant metastatic myxoid liposarcoma to the heart in a 54-year-old man, who presented with acute symptoms of cardiac tamponade. Surgery remains the best treatment option for this rare condition.
Key words: liposarcoma – myxoid – tamponade
Liposarcoma is the second most common malignant soft tissue tumor, exceeded only by malignant fibrous histiocytoma. Among the four pathological subtypes (well-differentiated, myxoid, round cell and pleomorphic), the myxoid variant is the most frequently encountered (1). Myxoid liposarcoma is characterized by long disease duration and multiple recurrences. Isolated myxoid liposarcoma cardiac metastasis is rare, with less than 10 cases reported in the English literature (2,3). We present a rare case of a rapidly progressing giant metastatic liposarcoma to the heart causing cardiac tamponade and discuss the diagnostic and management challenges.
CASE REPORT A 54-year-old man with good past health presented with a left chest wall mass in 1994. Wide local excision of the pectoralis muscle was performed and histology confirmed myxoid liposarcoma. Unfortunately, during the period between 16 months and 5 years after the excision, he suffered three recurrences locally in the left chest wall, and also developed new lesions at the left popliteal fossa and in the left thigh. These were managed by wide local excision and radiotherapy. Radiation therapy was used preoperatively as the tumor was adjacent to neurovascular bundles and to the bone where a marginal surgical margin was anticipated. Preoperative irradiation doses were to 50.4 Gy, delivered by twice daily fractionated treatments of 1.2 Gy. The patient remained asymptomatic and disease free radiologically for the next 2 years. In July 2001, he presented with a 2-week history of progressive
For reprints and all correspondence: Ahmed A. Arifi, Section of Cardiac Surgery, Division of Cardiothoracic Surgery, The Chinese University of Hong Kong, Prince of Wales Hospital, Sha Tin, NT, Hong Kong. E-mail:
[email protected]
shortness of breath, orthopnea and episodes of syncope at rest. Examination showed a mild tachycardia, elevated jugular venous pressure to the mandibular angle and a pan-systolic murmur over the left sternal border. There was no pericardial rub. Chest radiography revealed a widened mediastinum, markedly increased cardio-thoracic ratio and mediastinal shift to the right (Fig. 1a). An echocardiogram detected a huge mediastinum mass compressing on the left atrium and both ventricles. Computed tomography (CT) of the chest confirmed a hypodense, non-contrast-enhancing lesion in the antero-lateral mediastinum extending from the level of the aortic arch to the left hemidiaphragm, causing significant compression to the heart, left main bronchus and pulmonary artery and no pulmonary metastasis (Fig. 1b). Ultrasonography of the abdomen, radioisotope bone scan and CT of the brain were unremarkable. SURGERY Median sternotomy revealed a huge mass occupying almost the entire anterior mediastinum. The tumor was attached to the right ventricular outflow tract with a pedicle infiltrating into the right ventricular myocardium. En bloc resection was performed and the tumor pedicle was shaved from the myocardium. The patient made an uneventful recovery and was discharged 4 days post-operatively, with improvement of his symptoms. Radiotherapy was not offered owing to overlap with previous radiotherapy field. CT at 2 and 6 months followup showed no disease progression. PATHOLOGY The excised tumor was yellowish brown with a gelatinous consistency, weighed 1140 g and measured 18 ´ 14 cm. Microscopically, the mass was composed of proliferating small spindle cells and lipoblasts in the myxoid stroma, accompanied by a plexiform vascular network, compatible with myxoid liposarcoma (Fig. 2). No round cells or dedifferentiation were © 2002 Foundation for Promotion of Cancer Research
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INTRODUCTION
Jpn J Clin Oncol 2002;32(11)
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Figure 1. (a) Widened mediastinum and enlarged cardiac shadow on chest radiograph. (b) Computed tomography showing mediastinal mass compressing the heart, left pulmonary artery and lung.
DISCUSSION Cardiac metastasis from disseminated malignant neoplasm is a well-known phenomenon. However, in myxoid liposarcoma, isolated metastasis to the heart or pericardium is very rare with only a handful of cases reported in the English literature. In addition, the enormous size of the liposarcoma cardiac metastasis made this a unique case. The more common sites of metastasis for myxoid liposarcoma include the retro-peritoneum, abdominal cavity and chest wall (4–6). The reported time intervals between the appearance of the initial tumor and cardiac metastases were usually long, ranging from 7 to 25
Figure 2. Photomicrograph shows representative area of tumour consisting of proliferating small spindle cells and lipoblasts in the myxoid stroma, accompanied by plexiform vascular network (H&E, original magnification ´200).
years (7). In contrast, our patient had a relatively short lag period between the primary tumor and presentation of cardiac metastasis. Cardiac metastatic lesions can have a diverse clinical presentation, with the most common manifestation being congested heart failure, while others include conduction defects, mechanical obstruction, valvular disorders and pericardial disease. The signs and symptoms are associated with the direct effects of mechanical obstruction, tumor invasion into the myocardium causing impaired myocardial contraction or pericardial metastasis with effusion and tamponade (8). Dyspnea may be the main complaint when cardiac tamponade develops slowly. In general, most patients with metastatic disease to the heart remain asymptomatic for a long time, with the tumor often being an incidental post-mortem finding (9). For myxoid liposarcoma, the expansile rather than infiltrative growth pattern of the tumor usually causes symptoms relating to pressure
Figure 3. Two-dimensional echocardiogram shows the mass lesion and its relation to the heart.
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seen. Review of the pathological specimens from previous resections showed marked uniformity in histological appearance, suggesting metastasis.
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from symptomatic relief after surgery. Therefore, in isolated cardiac metastasis, surgical resection should be considered in selected patients for cure and palliation.
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on the heart or the surrounding large vessels. The rapidity with which our patient developed heart failure, dyspnea and syncope over a 2-week period was very unusual, especially when the chest radiograph taken 6 months prior to his symptoms was normal. Two-dimensional echocardiography should be the diagnostic tool of choice in suspected paracardial lesions. It can demonstrate intracavitary masses, pericardial effusion and myocardial compression and help assess ventricular function (10). In our case, the echocardiogram was useful in identifying the mass lesion and its relation to the heart; it also demonstrated the compression effect of the mass particularly to the filling of the heart in diastole (Fig. 3). It should be complemented with CT scanning and, in selected cases, MRI, which are better at delineating the size, location of the tumor and relation with adjacent structures and excluding mediastinal lymph node involvement that may preclude the patient from surgery (11). Historically, surgical intervention is rarely of benefit for secondary cardiac tumors. This is probably due to the poor control of the primary lesion and systemic dissemination that render resection futile (12). However, surgical ablation is still the best treatment option for isolated cardiac metastasis with reported survival of up to 14 years after complete resection, although the peri-operative mortality rate remains high (40%) (13,14). Unfortunately, our patient had significant myocardial invasion that rendered complete curative resection impossible. At follow-up 8 months after surgery, he was asymptomatic and a CT scan showed no evidence of disease recurrence. Despite the possibility of future cardiac recurrence, the patient benefited
