Primary Ewing sarcoma of the tentorium presenting with intracranial hemorrhage in a child.

Spontaneous intracerebral hemorrhage in children is usually related to cerebrovascular conditions. Brain tumors presenting with spontaneous bleeding account for approximately 10% of intracranial hemorrhages in children. The occurrence of primitive central nervous system lesions in the Ewing sarcoma family of tumors (ESFT) not related to bone or metastatic disease is a rare condition. The authors report on a child who presented with intracranial bleeding secondary to a nonmetastatic tentorial ESFT confirmed by detection of the fusion gene EWS-ERG. A detailed review of the literature reveals that most primary intracranial ESFT had a meningeal attachment, and that almost half of them presented at diagnosis with hemorrhage. Distinguishing between ESFT and other intracranial neoplasms is essential because the treatment and prognosis differ remarkably from that of other tumors, namely central primitive neuroectodermal tumors (PNETs). Whereas adjuvant treatment for ESFT consists of local or regional radiotherapy and chemotherapy containing alkylating agents, central PNETs are generally treated with whole neuraxis radiation and platinum-based chemotherapy. Additionally, the prognosis for intracranial ESFT might be better than the one for nonpineal central PNETs.