Metastatic Ewing's Sarcoma of the Occipital Squama Causing Non-Communicating Hydrocephalus: Case Report

Few reports of metastatic Ewing's sarcoma encroaching on the central nervous system have been available, and two types of CNS involvement have been described; one is invasion of the skull and/or dura forming an extracerebral mass, and the other is intraparenchymal infiltration causing focal symptoms. A 12-year-old male received a ventriculo-peritoneal shunt operation because of non-communicating hydrocephalus brought about by a metastatic bony tumor on the occipital squama two years after the amputation of the right humerus as an initial treatment for the primary Ewing's sarcoma. After an eight month interval of remission, he underwent a revision of the shunt but still neurological condition worsened. The associated soft tissue mass around his nuchal region exhibited a tremendously rapid growth. Three months later, he died of compression of the brain stem by the metastatic bony tumor occupying the cisterna magna. Though the post-mortem examination showed the cerebellum apparently demarcated from the mass, histological survey verified some invasion at the cerebellar surface which had had direct contact with the mass.