Discussion
Diagnosis With Brief Discussion
- Diagnosis
- Well differentiated mediastinal liposarcoma
- Radiologic Findings
- Initial chest radiograph demonstrates upper mediastinal widening with increased opacity at the level of aortic arch. Follow up chest PA after 45 months shows slightly progressed mediastinal widening. CT scan (mediastinal window) shows a fatty lesion with mass effect on superior vena cava and on the aortic arch. There is also increased volume of the mass at the same level of follow up chest CT. The mass composes largely of fatty tissue and some fine nodules and minimal streaky soft tissue portion.
- Brief Review
- Primary mediastinal liposarcomas are rare tumors that arise in the anterior and posterior mediastinum with relatively equal frequency. Less than 5% of liposarcomas arise in the mediastinum. Similar to primary retroperitoneal liposarcomas, mediastinal liposarcomas are most common in adults and are most often well differentiated.
The predominant finding of mediastinal liposarcoma on conventional chest radiography is a widened mediastinum. Deviation of the trachea and vessels may be apparent. On CT and MR imaging, mediastinal liposarcomas appear as inhomogeneous fatty masses that vary in appearance depending on the amount of soft tissue and fibrous bands in the tumor.
Well-differentiated liposarcomas or atypical lipomas are low-grade lesions containing a large amount of fat, usually more than 75%of their volume. They are difficult to confidently differentiate from benign lipomas. Fibrous septa may be broader and more nodular than those seen in lipomas. These tumors often recur if only marginally excised but do not metastasize.
Currently, the overall distribution of liposarcoma arising at all sites is 45% to 50% well-differentiated liposarcomas, 30% to 35% myxoid liposarcomas, 15% to 20% dedifferentiated liposarcomas, and 5% are pleomorphic liposarcomas. Overall, prognosis correlates with tumor subtype, as rapid tumor recurrence and metastatic disease were more common in patients with dedifferentiated and pleomorphic subtypes, whereas patients with well-differentiated tumors rarely recur or metastasize.
- References
- 1) Munk PL, Lee MJ, Janzen DL, et al. Lipoma and liposarcoma: evaluation using CT and MR imaging. AJR 1997;169:589-594
2) Hahn HP, Fletcher CD. Primary mediastinal liposarcoma: clinicopathologic analysis of 24 cases. Am J Surg Pathol. 2007 Dec;31(12):1868-74.
3) Munden RF, Nesbitt JC, Kemp BL, et al. Primary liposarcoma of the mediastinum. AJR 2000 Nov;175(5):1340
- Keywords
- mediastinum, malignant tumor,