...a case of recently described spindle cell tumor displaying a QKI-RAF1 gene fusion resistant to standard cytotoxic chemotherapy. The use of the mitogen-activated extracellular kinase (MEK) 1 and 2 inhibitor trametinib yielded clinical benefit and an absence of progression for 10 months...With secondary resistance to conventional multiagent chemotherapy, the use of the MEK1/2 inhibitor trametinib induced a prolonged metabolic response and clinical benefit.