SDPS-06 A 4 YEAR-OLD GIRL WITH EMBRYONAL RHABDOMYOSARCOMA IN RETROAURICULAR AND MIDDLE EAR : A RARE CASE REPORT FROM INDONESIA

Abstract Rhabdomyosarcoma is the most common sarcoma of childhood, and this tumor third neoplasm after neuroblastoma nephroblastoma. Rhabdomyosarcomas may originate in any anatomical site, occurring predominantly head neck regions, orbits, skull base, nasal cavity, nasopharynx, where there little or no musculoskeletal tissue. The involvement ear mastoid bone rhabdomyosarcoma uncommon. embryonal includes about 60– 70% cases. A four years-old girl presented with pain difficult to moving, was a mass left auricular that getting bigger. 4 days prior admission, drooping mouth left, voice sounds hoarse, but weakness side loss consciousness. On physical examination, N.VII palsy, without hyperreflexia, paraplegia, nystagmus. High resolution Mastoid CT-Scan showed characteristic malignant solid involving external acoustic canal tympanic cavities, destroying auditory temporal bones, internal canal, reaching extending into intracranial region cerebellopontine angle duramater. In Pathology examination Tumor cells are spherical, spindle-shaped pleomorphic nuclei, hyperchromatic, coarse chromatin, vesicular, some real nuclear daughters, eosinophilic cytoplasm. Mitosis discovered. eccentricallynucleated cytoplasm, suggesting rhabdomyoblast differentiation, "tadpole" appearance, conclusion according rhabdomyosarcoma. patient treated chemotherapy using Vincristine, Actinomycin-D, Ifosfamide.