Primary intracranial rhabdomyosarcoma producing proptosis SAMRUAY SHUANGSHOTI AND CHARE PHONPRASERT

SYNOPSIS A case is reported of a primary rhabdomyosarcoma occurring in the right subfrontal region of a 16 year old girl. The patient suffered from dull frontal headache and proptosis for three months before hospitalization. The circumscribed and demarcated neoplasm involved the dura mater, and invaded the frontal sinus and roof of the orbit on the same side. The patho-genesis of the tumour is thought to be related to aberrant differentiation of unstable mesenchyme. A suggestion is made that the 'medullomyoblastoma ' should be classified as a type of neoplasm of mixed mesenchymal and neuroepithelial origin. Primary neuraxial sarcomas are rare. Butler and Netsky (1973) in reviewing nine reported series containing 27 487 intracranial neoplasms found only 423 primary sarcomas (1.5%/o)