Most leiomyosarcomas of the mediastinum arise from the walls of the esophagus or great vessels leading to the clinical signs and symptoms of esophageal obstruction or superior vena cava syndrome. Primary mediastinal leiomyosarcomas unassociated with these structures are extremely uncommon. Their origin is believed to be the small vessels of the mediastinal soft tissue, heterotopic smooth muscle displaced during embryonic development, or a parasitic process in which the tumors become detached from the walls of the esophagus during their growth. Distinction of the exact site of origin is important in order to determine the extent of surgery which will have to be more extensive if the esophagus or great vessels need to be removed. In rare instances, mediastinal leiomyosarcomas may form part of a teratomatous lesion that has undergone sarcomatoid differentiation. Ample sampling of any mediastinal lesions is always important to exclude such a possibility (Moran et al. 1994;...
References and Further Reading
- Villacis, R. A., Silveira, S. M., Barros-Filho, M. C., Marchi, F. A., Domingues, M. A., Scapulatempo-Neto, C., Aguiar, S., Jr., Lopes, A., Cunha, I. W., & Rogatto, S. R. (2014). Gene expression profiling in leiomyosarcomas and undifferentiated pleomorphic sarcomas: SRC as a new diagnostic marker. PLoS ONE, 9, e102281.PubMedPubMedCentralCrossRefGoogle Scholar