Primary Intracranial pPNET/Ewing Sarcoma: Diagnosis, Management, and Prognostic Factors Dilemma-A Systematic Review of the Literature.

Abstract

Ewing sarcoma (ES) is a malignant bone tumor that is most commonly observed in the long bones, the pelvis, and the chest. Primary intracranial localization is extremely rare. In the aim of identifying the prognostic factors of this rare localization, we conducted a systematic review of the literature for patients with primary intracranial peripheral primitive neuroectodermal tumor/ES. A complete MEDLINE search was undertaken for all articles reporting data for primary intracranial peripheral primitive neuroectodermal tumor/ES. We divided the patient population into 2 groups: group I, patients who were free of disease; and group II, patients who died or had uncontrolled disease at the last available follow-up. We conducted a comparative statistical analysis of data between the 2 groups. A total of 48 patients were included in the review. There were 32 patients in group I and 12 in group II. Fifty percent of tumors in group II and only 16% of tumors in group I were infratentotorial (P = 0.03). Signs of bone involvement were observed in 19% of patients in group I and 54% in group II (P = 0.03). Total removal was accomplished in 29% of patients in group I and in no patients in group II (P = 0.03). Radiotherapy was performed in 73% of patients in group II and 81% in group I (P = 0.43). Chemotherapy was administered in 36% of patients in group II and 74% in group I (P = 0.03). Infratentorial localization and the presence of bone involvement were associated with poor prognosis; Surgery seems to be a predictive factor of prognosis; radiotherapy and chemotherapy must be performed whenever the tumor is not totally removed.