Abstract
Introduction. Inflammatory myofibroblastic tumors (IMTs) are rare soft tissue neoplasms that occur predominantly in young adults. IMTs occur most commonly occur in the lungs, are localised and have a favourable prognosis. The pathogenesis of IMTs is based on translocation of the ALK gene in more than half of the cases.
Case description. This article presents two clinical observations of the use of the ALK inhibitor crizotinib in young patients with advanced extrapulmonary IMTs with ALK gene alterations. In both cases, the duration of response to therapy was more than 10 years. In the first observation, a patient with inoperable IMT achieved disease stabilisation while receiving targeted therapy. In the second case, a patient with multiple lung, bone and lymph node metastases achieved a partial response to crizotinib therapy at the first follow-up evaluation, which persisted thereafter. In both cases, the therapy was well tolerated and no new adverse events were observed.
Conclusion. The use of the ALK inhibitor crizotinib in the advanced form of IMTs with ALK translocation made it possible to achieve long-term responses to the therapy (more than 10 years).
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