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J Thorac Cardiovasc Surg 2005;129:1440-1442
© 2005 The American Association for Thoracic Surgery
Brief Communication |
Division of Cardiac Surgery, University "G D’Annunzio," Chieti-Pescara, Chieti, Italy.
Received for publication December 10, 2004; accepted for publication January 10, 2005. * Address for reprints: Gabriele Di Giammarco, MD, Chief of Division of Cardiac Surgery, University "G D’Annunzio," S Camillo Hospital, v Forlanini 50, 66100 Chieti, Italy (Email: gabriele.digiammarco1{at}tin.it).
Myxoid liposarcoma is a very uncommon primary neoplasm in the mediastinum, representing 9% of all primary sarcomas in this area.1,2 It develops preferentially in the lower extremities (75%) and less frequently in the retroperitoneum.1 Myxoid liposarcoma usually metastasizes in the lungs and bone,1 and rarely in the mediastinum.3 The time interval between the first presentation and the pericardial metastasis ranges from 7 to 25 years.1 A case of giant metastatic myxoid liposarcoma in the pericardium causing cardiac tamponade is reported.
Clinical Summary
A 62-year-old man underwent 3 resections of a primary myxoid liposarcoma of the left thigh in 1983, 1985, and 1989. He was admitted to the hospital with reports of dyspnea for the previous 3 weeks and chest pain of recent onset. The computed tomography (CT) scan performed 6 months previously at a follow-up examination did not show any metastasis. No abnormalities were evident on the electrocardiogram. The chest radiography revealed a widened mediastinum. Echocardiography showed a big mass in the pericardium compressing the right sections of the heart. The CT scan confirmed the presence of a mediastinal mass with dimensions of 23.5 x 18.0 x 15.0 cm (Figure 1). On the same day, the patient underwent surgical resection.
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The patient had an uneventful postoperative recovery and was discharged on the fifth postoperative day. The first CT did not show any recurrence of the tumor.
The resected mass measured 21.0 x 17.0 x 15.0 cm and weighed 2430 g. It was yellowish and mainly encapsulated except for the infiltrating area. The cut surface revealed myxoid and necrotic areas. Microscopy showed prevalent myxoid stroma containing lipoblasts and myocytes in the infiltrating area (Figure 2). It was labeled grade I, with the percentage of round cells less than 10%.1
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Wong and colleagues4 reported a case of a 54-year-old patient with a previously resected liposarcoma of the left pectoralis muscle who underwent operation for a metastatic tumor located in the anterior mediastinum and measuring 18 x 14 cm. They stated that the excised mass was the biggest mediastinal metastatic myxoid liposarcoma ever reported.
Myxoid liposarcoma has an expansive rather than infiltrative growth, so it can cause symptoms because of compression of the heart and great vessels.4 Symptoms are dyspnea, wheezing, and chest pain, even if the tumor grows asymptomatically.2
In our case, the patient stayed asymptomatic until 3 weeks before surgery, with a negative CT scan 6 months before. This means the described metastasis occurred long after the first presentation of the primary tumor (21 years), but developed in less than 6 months.
Although the operative mortality ranges from 12% to 40%,2,5 surgical resection remains the only chance in these rare cases.
Patients with myxoid liposarcoma who are surgically treated demonstrate good survival even 10 years after surgery. Multivariate analysis individuated the presence of round cells, age (>45 years), and necrotic areas as independent variables for a lower survival at 10 years; the grade of the neoplasm influences the incidence of metastasis and its recurrence.1
Patients with deep-seated liposarcoma more frequently have recurrences within 6 months; the main negative factor is an incomplete resection.1
In our case, evidence of necrotic areas within the mass and age more than 45 years have to be considered unfavorable prognostic factors, but the low-grade round cells differentiation and complete resection of the tumor could provide a good survival free of recurrences.
Conclusions
Our case can be considered unique for the giant dimensions and rare location of the metastasis. Complete surgical resection represents the only chance to improve survival and quality of life in these rare cases.
References
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