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The patient had an acute episode of IVA causing severe metabolic stress and eventually died. A new case of an IVA patient carrying c.1193GA (p.Arg398Gln) and c.1208AG (p.Try403Cys) mutations is reported in China. G (p.Try403Cys) mutations is reported in China. Inflammatory myofibroblastic tumor (IMT) is a distinct tumor with a low incidence rate, which can be diagnosed at any age with a predilection for children and adolescents. Although IMT is visible in any tissues and organs, it is more commonly found in the lungs. The clinic