Recent progress in the classification of soft tissue tumors: role of genetics and clinical implications

Soft tissue tumors comprise a vast and heterogeneous group of neoplasms. Because different tumors often have different biological behaviors and respond differently to various therapeutic modalities, precise classification is paramount. The majority of soft tissue tumors were first delineated on the basis of morphologic and clinical findings, which in many cases were adequate to accurately separate different tumors into homogeneous groups; however, it has increasingly been appreciated that many entities are actually heterogeneous groups of tumors that have similar histologic and pathologic characteristics but differ in their clinical behavior and underlying pathogenesis. Within the past several years, great strides have been made in the purification of different entities. This accomplishment has largely been because of advances in our understanding of the molecular genetics that underlie the pathogenesis of many sarcomas and the development of new and specific tumor markers. This review highlights some important recent work in two selected soft tissue tumors-gastrointestinal stromal tumor and inflammatory myofibroblastic tumor. These examples illustrate the type of progress that is being made in the classification of soft tissue tumors.