Rhabdomyosarcoma of the Middle Ear Case Report.

BACKGROUND

Rhabdomyosarcoma (RMS) is a highly malignant soft tissue tumor derived from primitive embryonal mesenchymal tissue that differentiates into striated skeletal muscle. Despite the improved outcome based on the EFS and OS using the three different treatment modalities-chemotherapy, radiotherapy and surgical treatment, the survival of patients depends on their IRS groups-pathological and surgical. On the other hand in the last thirty years a great improvement of the five-year overall survival (OS) of children with RMS have been observed based on the results of large multinational collaborative trials and successive studies dedicated to children, though prognosis is variable and dependent on several factors including histologic variant, primary sites of the tumor, extent of disease (disease resectability), and molecular-level characteristics.

CASE PRESENTATION

We present a clinical case of a five-year-old male with initial complains of left side peripheral facial nerve palsy and secondary cervical and retroauricular lymphadenomegaly. After an exam, surgery of the temporal bone, CT and MRI embryonal type of rhabdomyosarcoma was diagnosed, and adjuvant chemotherapy was initiated in combination with concomitant local radiotherapy.

RESULTS

The results show that in these areas surgery itself is insufficient for RMS treatment(usually it is limited to taking a biopsy only). The combination of chemotherapy and local control with radiotherapy achieved a good result in our patient.

CONCLUSIONS

Middle ear Embryonal Rhabdomyosarcoma is a common solid tumor, which could mimic middle ear inflammation or mastoid inflammation in patients. The multimodal approach seemed to be the ideal management of RMS. It involves a combination of chemotherapy and local control with surgery and/or radiotherapy.