This review consolidates information gleaned from several case reports and larger series on Ewing's sarcoma family of tumors (EFT) involving structures related to and found in the central nervous system (CNS). These tumors involve the skull, the spinal column, adjacent soft tissues, the meninges, and the brain. We have separated the cases by skull region and spinal column level, and we discuss the attendant differences in prognosis following treatment by neurosurgery, radiation, and chemotherapy. Light and electron microscopic features can be used to differentiate EFT from other small round blue cell tumors that involve the CNS (central primitive neuroectodermal tumor, lymphoma, etc.). Recent molecular and genetic findings in EFT provide new diagnostic methods. We conclude that EFT involving the CNS and adjacent structures is not so rare as previously stated and that the prognosis is more favorable, as a rule, than for the more common examples arising in the long bones and pelvis.

A 1-year-old female infant presented with a large 7–8 cm Ewing’s sarcoma of the left parietal calvarium. It deeply indented the subjacent hemisphere. Wide surgical excision was followed by chemotherapy but not radiation. The patient was clinically free of tumor when she expired 9 months later of sepsis. Though primary Ewing’s sarcoma of the skull is said to be relatively rare, we have tabulated 37 cases of this disorder, including the present one (we excluded mandibular tumors (>100 cases) since they are not usually treated by neurosurgeons). The mean age was 11 years and of those available to follow-up, three quarters were tumor-free 6 months to 7 years postsurgery. The remainder survived an average of 21 months. Ewing’s tumors of the calvarium generally grant a favorable prognosis following surgery and/or radiation and chemotherapy. Those at the base of the brain may be more difficult to eradicate. In addition to primary Ewing’s tumors, metastases to the skull and the brain from other primary sources in the skeleton have been documented in several reports.