Liposarcomas of the mediastinum and thorax: a clinicopathologic and molecular cytogenetic study of 24 cases, emphasizing unusual and diverse histologic features.

摘要: Liposarcoma rarely occurs in the mediastinum, and most reports predate current genetically based classification system. We report clinicopathologic molecular genetic features of a series thoracic liposarcomas identified over 60-year period. Twenty-four confirmed cases were reclassified using recent World Health Organization classification. Fluorescent situ hybridization for CPM amplification and/or DDIT3 rearrangement was performed on selected cases. The 24 occurred 13 men 11 women (mean age, 53 y; range, 15 to 73 y) arose all mediastinal compartments. All subtypes encountered with 8 well-differentiated liposarcomas, 6 dedifferentiated (3 CPM+), 7 pleomorphic (2 CPM-, 1 DDIT3-), 2 myxoid unclassifiable liposarcoma (CPM- DDIT3-). Unusual histologic included mimicking cases), lipoleiomyosarcoma (1 case), "meningothelial"-like dedifferentiation, differentiated (CPM-), epithelioid change. Follow-up information available 19 patients (mean, 55 mo; 252 mo). Outcome strongly associated subtype, death from disease occurring well-differentiated, 4 dedifferentiated, 5 pleomorphic, liposarcomas. mediastinum shows preponderance uncommon unusual morphologic variants. Correct has important implications, well-differentiated/dedifferentiated having protracted clinical course, contrast more rapid progression seen liposarcoma.