TY - JOUR AU - Sarmiento, Daniel E. AU - Clevenger, Jessica A. AU - Masters, Gregory A. AU - Bauer, Thomas L. AU - Nam, Brian T. PY - 2015 TI - Epithelioid inflammatory myofibroblastic sarcoma: a case report JF - Journal of Thoracic Disease; Vol 7, No 10 (October 31, 2015): Journal of Thoracic Disease Y2 - 2015 KW - N2 - Inflammatory myofibroblastic tumor (IMT) of the lung is a rare malignancy with few cases reported in the literature. Histologically, it is composed by spindle cells and an infiltrate of inflammatory cells. Children and young, non-smoking adults constitute the majority of cases, the clinical behavior ranges from a benign entity to a malignant process with rapid recurrence and metastatic progression. We present a case of epithelioid inflammatory myofibroblastic sarcoma (EIMS) of the pleura, a malignant variant of IMT, which was initially treated with debulking surgical resection followed by systemic chemotherapy. The tumor was found to have an anaplastic lymphoma kinase ( ALK ) gene rearrangement. An ALK directed tyrosine kinase inhibitor was used with an impressive response, the patient remains in remission nearly 1 year after presentation. The pathogenesis, pathologic findings, clinical behavior and imaging of pulmonary EIMS are discussed. UR - https://jtd.amegroups.org/article/view/5598