Sustained complete response on crizotinib in primary lung inflammatory myofibroblastic tumor - Case report and literature review

Abstract

Inflammatory myofibroblastic tumors (IMT) are rare soft tissue tumors of intermediate malignant potential occurring usually in children and adolescents. Treatment options for advanced diseases are limited. A 35-year-old lady presented to us with fever, cough and decreased appetite. On evaluation, she was diagnosed with left lung IMT. She underwent surgery and developed recurrence with pleural nodules after two years. Immunohistochemistry showed positivity for ALK (diffuse). Since recent evidence suggested that crizotinib is effective in advanced IMT with 50% response rates, she was treated with crizotinib 250mg BD with which she had a complete radiological response at three months. She has completed one year of treatment thus far and continues to be in complete remission. Treatment with ALK inhibitors like crizotinib has brought about a paradigm shift in the management of advanced ALK-positive IMT’s with excellent clinical responses which are durable in a majority of cases.