What is the prognosis of malignant mesenchymal tumors in the mediastinum?

Updated: Feb 16, 2021
  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: John Geibel, MD, MSc, DSc, AGAF  more...
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Answer

The prognosis for liposarcoma depends on several factors. Completely resected pseudoencapsulated tumors have a better prognosis compared to those that are nonencapsulated and invasive. Cell type and cell differentiation also play a role. Myxoid liposarcoma has a poorer prognosis. In one small series, approximately 30% of patients died of their disease after a mean period of less than 3 years.

Angiosarcomas of the mediastinum are rare but have a very poor prognosis, especially when they originate in the heart or great vessels.

Fibrosarcomas have a uniformly poor prognosis, and most patients die from their disease within a few years.

Primary leiomyosarcoma of the mediastinum has been described. In one reported series of 10 patients who underwent resection, two were alive and well at 4 and 6 years. Four of the others died of their disease or developed recurrence.

Rhabdomyosarcoma has the best long-term survival of all mesenchymal tumors because effective chemotherapeutic regimens have been identified. According to one report, 10-year actuarial survival rates range from 52% to 83%, depending on the stage of disease at presentation and the existence of hematogenous metastases. Survival is much worse if the latter is present.


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