Inflammatory Myofibroblastic Tumour: State of the Art

Simple Summary Among sarcomas, which are rare cancers, inflammatory myofibroblastic tumors are extremely rare. Unlike other subtypes, this is a largely oncogene-driven neoplasia, and early gene rearrangement identification is important for accurate advanced stage treatment. In this manuscript, we review the clinicopathologic characteristics of this ultra-rare entity, as well as the current treatment landscape, with a particular focus on opportunities provided by tyrosine kinase inhibitors (TKIs). An inflammatory myofibroblastic tumor (IMT) is a neoplasm composed of myofibroblastic and fibroblastic spindle cells accompanied by inflammatory cells, including lymphocytes and eosinophils. It is an ultra-rare tumor, the optimal management of which remains to be defined. Surgery is the treatment of choice for localized tumors. The treatment of advanced disease is not precisely defined. Chemotherapy regimens result in an overall response rate of approximately 50% based on retrospective data. The latest pathophysiological data highlight the role played by tyrosine kinase fusion genes in IMT proliferation. Anaplast lymphoma kinase (ALK) oncogenic activation mechanisms have been characterized in approximately 80% of IMTs. In this context, data regarding targeted therapies are most important. The aims of this article are to review the latest published data on the use of systematic therapy, particularly the use of molecular targeted therapy, and to publish an additional case of an IMT with Ran-binding protein 2 (RANPB2)-ALK fusion showing a long response to a tyrosine kinase inhibitor.