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.

Extraskeletal Ewing’s sarcoma (EES) is a rare tumor arising from the soft tissue. It commonly involves the paravertebral region followed by lower extremities, chest wall, retroperitoneum, pelvis and hip, and upper extremities. We report a case of EES located in the distal phalanx of the right thumb in a female patient aged 17 years. Considering the infrequency of extraskeletal type and site, as in this case, we highlight the rarity of EES of the thumb as well as the importance of histopathology in diagnosing these rare cases to provide proper treatment to the patient.