Abstract
Within the last decade, advances in tumor cytogenetics and molecular biology have led to an explosion of information related to the underlying molecular alterations in sarcomas. Many sarcomas have characteristic genetic findings that shed light on mechanisms of tumorigenesis, serve as diagnostic and/or prognostic markers, give clues to relationships between different sarcomas, and reveal targets for biologically rational therapies. This review summarizes recent progress in this fast-moving field, with particular emphasis on newly characterized genetic alterations present in inflammatory myofibroblastic tumor, alveolar soft part sarcoma, lipoblastoma, and angiomatoid fibrous histiocytoma. Also emphasized are developments in “genetic” diagnostics, new insights into the relationships between different entities, prognostic implications of genetic alterations, the development of an animal model for liposarcoma, and the validation of a rational therapy for gastrointestinal stromal tumors.
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