부산대학교 도서관

Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood, representing 5% of all childhood cancers. We are reporting an interesting case of parameningeal rhabdomyosarcoma in a child who underwent multimodal treatment. A 7-year-old boy presented to our clinic with a history of bad breath, nasal obstruction and recurrent epistaxis from the left nostril for 3 months. On examination he had mild left proptosis with normal eye vision and movements, reddish left nasal mass with a smooth surface. Paranasal CT scan showed a slightly enhancing soft tissue mass 72 × 77mm in the nasal cavity that deviated the nasal septum to the right, extending to the nasopharynx posteriorly and to the maxillary and ethmoidal sinuses with another mass 11 × 16mm extension into the orbit. Biopsy was taken and histology showed embryonal rhabdomyosarcoma. An extensive tumour debulking was done followed by chemotherapy (vincristine, dactinomycin and cyclophosphamide) and 50 grays of radiotherapy. A surveillance CT scan a month after treatment revealed over 90% tumour reduction. All patients with metastatic disease (group IV, stage 4) are considered high risk, except children and adolescents younger than 14 years with embryonal rhabdomyosarcoma. Advances into the multimodality management have dramatically improved survival in PM-RMS from approximately 25% to 75%.