The frequency of isolated CNS involvement in Ewing's Sarcoma

Abstract

Records of 161 patients with Ewing's sarcoma who were treated at the National Cancer Institute and 284 patients entered into the Intergroup Ewing's Sarcoma Study I were reviewed to assess the incidence of central nervous system (CNS) involvement in patients with Ewing's sarcoma. The total incidence of central nervous system involvement was 10/445 (2.2%). Intraparenchymal or meningeal central nervous system involvement without adjacent bony or dural involvement occurred in 7/161 patients (4.3%) at the National Cancer Institute and 3/284 (1.1%) patients in the Intergroup study. Cortical mass lesions were found in 8/445 (1.8%) patients and meningeal involvement in 2/445 (0.4%). There were no patients with Ewing's sarcoma who had central nervous system involvement documented at presentation. In seven of eight patients with intraparenchymal cortical disease there was evidence of other metastases or a history of at least one previous relapse at the time of the CNS involvement with Ewing's sarcoma. Four of the seven National Cancer Institute patients in whom central nervous system involvement occurred were part of a larger group of 93 patients treated with prophylactic central nervous system irradiation and intrathecal methotrexate. An occipitoparietal mass and pulmonary metastases developed in one of these patients five years after receiving treatment. In three of the ten patients, meningeal (two) or intraparenchymal CNS (one) involvement was the initial site of tumor recurrence. The data indicate that isolated meningeal or intraparenchymal central nervous system involvement in Ewing's sarcoma is uncommon. These results do not support the necessity for central nervous system prophylaxis in Ewing's patients with sarcoma. Copyright © 1982 American Cancer Society