We report a case of embryonal rhabdomyosarcoma originating in the right ethmoidal sinus. A 68-year old man reporting right cheek swelling and epiphora for 2 months was found in computed tomography to have a tumor in the right ethmoidal sinus and bilateral neck lymph nodes metastasis. Bone scintigraphy showed metastasis to the ribs. We treated him with irradiation and chemotherapy. The chemotherapy protocol was CYVADIC (cyclophosphamide, vincristine, adriamycin, dacarbazine). He underwent chemotherapy 5 times but the tumor was not controlled. Imatinib mesilate (Glivec®) is a new drug targeting mutations of the c-kit gene that can have therapeutic effects in gastrointestinal stromal tumors and leukemia. We tried imatinib mesilate because the tumor showed positive staining for c-kit. The drug was not effective, but gene-targeting drugs such as imatinib mesilate may be useful for patients with malignant tumors because there are many such c-kit positive tumors.
