EPITHELOID INFLAMMATORY MYOFIBROBLASTIC SARCOMA AND RESPONSE TO CRIZOTINIB

Jpn J Clin Oncol. 2014 Sep;44(9):868-71. doi: 10.1093/jjco/hyu069. Epub 2014 Jul 15.

A Case Report of Epithelioid Inflammatory Myofibroblastic Sarcoma with RANBP2-ALK Fusion Gene Treated with the ALK Inhibitor, Crizotinib.

Kimbara S1, Takeda K1, Fukushima H2, Inoue T3, Okada H1, Shibata Y1, Katsushima U1, Tsuya A1, Tokunaga S1, Daga H1, Okuno T2, Inoue T2.

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Abstract

Epithelioid inflammatory myofibroblastic sarcoma is a variant of inflammatory myofibroblastic tumor with aggressive clinical course associated with RANBP2-ALK fusion. The present report describes a case of a 22-year-old Japanese man with a pelvic mesenchymal neoplasm. The feature of the neoplasms, including epithelioid morphology, anaplastic lymphoma kinase staining on the nuclear membrane, and results from the reverse transcriptase-polymerase chain reaction, led to diagnosis of epithelioid inflammatory myofibroblastic sarcoma with RANBP2-ALK fusion. Despite two surgical excision procedures, local recurrence rapidly occurred, and the tumor developed resistance to conventional chemotherapy with doxorubicin. Subsequent administration of crizotinib, an oral anaplastic lymphoma kinase inhibitor, resulted in tumor shrinkage. Distinguishing epithelioid inflammatory myofibroblastic sarcoma from conventional inflammatory myofibroblastic tumor is important, and crizotinib is a promising treatment for this aggressive tumor.