Rhabdomyosarcoma

ICD-O:8900/3

Rhabdomyosarcoma is seen occasionally in the middle ear of young children [126]. On rare occasions it is found in the middle ear of adults [81]. The tympanic membrane is usually eroded by the growth, which extends into the external canal. Grossly, the tumour is lobulated and dark red with a haemorrhagic cut surface. Almost all temporal bone rhabdomyosarcomas are of the embryonal type, displaying mainly spindle or round primitive skeletal muscle cells, some of which have clear cytoplasm staining positively for glycogen and others have eosinophilic areas in the cytoplasm. Cross striations are unusual in this neoplasm. Immu-nohistochemical markers for desmin, muscle-specific actin and antibodies against MyoD1 and myogenin confirm this diagnosis.

Rhabdomyosarcoma of the temporal bone is highly malignant and spreads extensively into the cranial cavity, externally or to the pharyngeal region. Lymph node and bloodstream metastases frequently develop in these patients.

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