Primary cerebral lymphomas: unsolved issues regarding first-line treatment, follow-up, late neurological toxicity and treatment of relapses. The FNCLCC. French Fédération Nationale des Centres de Lutte contre le Cancer.

BACKGROUND: Primary cerebral non-Hodgkin's lymphomas (NHL) in immunocompetent patients (PCL) are located exclusively in the central nervous system, the eye, or meninges. Clinical management of these patients remains controversial. PATIENTS AND METHODS: Clinical characteristics of the patients and parameters influencing their outcome as of December 1998 were investigated and registered in a database of 226 patients treated in the French Federation of Cancer Centers between 1980 and 1995.
RESULTS: Most PCL are diffuse large-cell NHL with a B phenotype. The incidence of PCL has been steadily increasing over the past 20 years in some but not all countries. The overall survival of primary cerebral lymphoma (PCL) patients in the published series, a median of 12-16 months and a five-year survival of 5%-20%, is poor. Several series have now reported long-term survivals of more than 10 years and PCL may therefore be a curable tumor in some patients. The optimal treatment of PCL is not known. Complete resection of the tumor does not improve outcome and multidisciplinary approaches combining chemotherapy and radiotherapy are now commonly used, although the superiority of combination over radiotherapy- or chemotherapy-alone has never been demonstrated in a phase III trial. The optimal chemotherapy regimen, the dose and even the usefulness of brain radiotherapy after chemotherapy are therefore still matters of debate. Recently, several authors have reported a relatively high incidence of late neurological sequelae after PCL treatment.
CONCLUSIONS: The optimal treatment of PCL patients remains to be defined. Large cooperative international phase III trials are now required to define and improve the optimal treatment of PCL and reduce its sequelae.