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Winthrop University Hospital Mineola, NY 11501
A 56-year-old obese woman was admitted to our institution for an open biopsy and thoracic exploration of a known mediastinal mass. On admission, she complained of 6 months of shortness of breath, worsening dyspnea on exertion, and a nonproductive cough. The patient also had signs of superior vena cava syndrome. Besides a history of smoking (30 pack-year), her medical history and surgical history were unremarkable.
A chest radiograph revealed mediastinal widening that appeared unchanged from that shown on radiographs obtained 5 years earlier. Even in retrospect, the chest radiographs obtained at admission clearly showed no change. A CT scan of the thorax, which had been obtained at another institution 5 years earlier to evaluate the mediastinal widening, revealed only prominent mediastinal fat. A CT scan obtained just before the current admission showed, in addition to the mediastinal lipomatosis, a new complex mediastinal mass (Fig. 1A, 1B). The mass was composed primarily of solid tissue in the anterior right mediastinum, which was compressing the superior vena cava, and of a combination of solid tissue and fat posterior to the right pulmonary artery.
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The patient underwent a right Chamberlain procedure with biopsy of the mass. At surgery, the mass was determined to be unresectable, although the anterior mediastinal component was debulked. Pathologic examination of the biopsy specimen, obtained from the anterior mediastinum, revealed a highgrade sarcoma consistent with a pleomorphic liposarcoma. An additional anterior mediastinal biopsy sample obtained more inferiorly—that is, roughly corresponding to the level shown in Figure 1B (where no soft-tissue mass is seen and mediastinal lipomatosis appeared unchanged)—confirmed the presence of coexistent benign fat.
Primary liposarcomas of the mediastinum are relatively rare, and to our knowledge, no cases associated with preexistent mediastinal lipomatosis have been reported. The presence of mediastinal lipomatosis (as opposed to a normal amount of mediastinal fat) was most likely coincidental in our patient with a mediastinal liposarcoma, although reliance on the serial chest radiographs alone would have masked the correct diagnosis. Malignant mesenchymal neoplasms of the mediastinum represent approximately 6% of primary mediastinal tumors, with liposarcoma as the most common type [1]. Mediastinal liposarcomas are often large and most commonly arise in the anterior mediastinum. Patients may be symptomatic—with dyspnea, tachypnea, wheezing, chest pain, cough, weight loss, and superior vena cava syndrome—or asymptomatic with the diagnosis made after the detection of a mass on chest radiography performed for other reasons [1, 2].
On CT, the appearance of mediastinal liposarcomas, as of liposarcomas elsewhere, varies from a predominantly fat-containing mass to a solid mass [2, 3]. The differential diagnosis includes mediastinal lipoma, thymolipoma, and teratoma [1].
The clinical course of mediastinal liposarcomas has been characterized by frequent recurrences and death, although metastatic spread is extremely rare [1, 4]. Treatment is surgical resection whenever possible, with radiation therapy possibly benefiting patients with recurrent or unresectable disease [1]. Surgery may be complicated by many factors including involvement by tumor of major vessels and difficulty determining tumor margins because of the gross similarity of liposarcoma to normal fat [1]. Debulking of tumor can be beneficial, however, in patients suffering from compressive symptoms [1].
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  M. Kara, M. Ozkan, S. Dizbay Sak, and S. Kavukcu Successful removal of a giant recurrent mediastinal liposarcoma involving both hemithoraces Eur. J. Cardiothorac. Surg., September 1, 2001; 20(3): 647 - 649. [Abstract] [Full Text] [PDF]
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