Epithelioid inflammatory myofibroblastic
sarcoma (EIMS) is an aggressive subtype of inflammatory myofibroblastic
tumor (IMT) harboring
anaplastic lymphoma kinase (ALK) gene fusions and is associated with high risk of local recurrence and poor prognosis. Herein, we present a young, non-smoking male who presented with complaints of
cough and dyspnoea and was found to harbor a large right lower lobe lung mass. Biopsy showed a high-grade epithelioid to
rhabdoid tumor with ALK and
desmin protein expression. The patient initially received 5 cycles of
crizotinib and remained stable for 1 year; however, he then developed multiple bony
metastases, for which complete surgical resection was performed. Histopathology confirmed the diagnosis of EIMS, with ALK gene rearrangement demonstrated by fluorescence in situ hybridization. Postoperatively, the patient is asymptomatic with stable metastatic disease on
crizotinib and has been started on palliative
radiotherapy. EIMS is a very rare subtype of IMT that needs to be included in the differential diagnosis of ALKexpressing lung
malignancies in young adults.