Embryonal rhabdomyosarcoma of the ear: a review of the literature and case history.

Approximately 50 per cent of all rhabdomyosarcomas in children occur in the head and neck region with the orbit, nasopharynx and ear in order of descending frequency. Embryonal rhabdomyosarcoma is the commonest malignant tumour of the aural region in childhood and its clinical course is usually rapidly fatal, with extensive local disease and or distant metastases (Dehner and Chen, 1978). Other malignant tumours that can occur in children include melanoma and other mesenchymal tumours, including undifferentiated sarcoma, fibrosarcoma, osteogenic sarcomas and Ewing sarcoma. Secondary extension may occur from a meningioma. Osseous disorders of the temporal bone, such as eosinophilic granuloma and Hand-Schüller-Christian disease, should be included as a differential diagnosis (Lewis, 1979).