Epithelioid inflammatory myofibroblastic sarcoma with VCL-ALK fusion of central nervous system: case report and brief review of the literature

Epithelioid inflammatory myofibroblastic sarcomas are an aggressive variant of inflammatory myofibroblastic tumor described primarily in the abdomen and less commonly in pulmonary location. The anaplastic lymphoma kinase (ALK) fusion partners described in this tumor include RANB2, RRBP1 and EML4. While rare examples of inflammatory myofibroblastic tumors have been described in the central nervous system, the epithelioid variant has never been described. The ALK-VCL fusion has been described in renal cell carcinoma, high-grade glioma and epithelioid fibrous histiocytoma but has not been described in epithelioid inflammatory myofibroblastic sarcoma or even inflammatory myofibroblastic tumor. Herein, we report the first case of epithelioid inflammatory myofibroblastic sarcoma in the central nervous system as well as the first case with VCL as the fusion partner for ALK.