Aim:Gliomatosis cerebri(GC)is defined as a rare pattern of growth of diffuse gliomas involving three or more cerebral lobes.Given its rarity,it is difficult to define prognostic factors and standard of treatment.We re...Aim:Gliomatosis cerebri(GC)is defined as a rare pattern of growth of diffuse gliomas involving three or more cerebral lobes.Given its rarity,it is difficult to define prognostic factors and standard of treatment.We retrospectively analyzed patients(PT)with GC from a single institution with the aim of identifying the main prognostic factors and to assess optimal management.Methods:Medical records were reviewed of patients≥18 years with a histological and/or radiological diagnosis of GC(with no contrast enhancement)occurring between 2006 and 2017.Median progression free survival(PFS)and overall survival(OS)were calculated by the Kaplan-Meier method.Results:We analyzed 33 PT,22 males and 11 females;Eastern Cooperative Oncology Group(ECOG)performance status(PS)was 0-1 in 21 of the patients.Twenty-two PT underwent biopsy:16 were astrocytomas and 6 oligodendrogliomas.O6-methylguanin-DNA-methyltransferase(MGMT)was detected in 14 cases,and it was methylated in eight cases.Isocitrate dehydrogenase 1(IDH1)was analyzed in 16 PT,and it had mutated in 10 of them.Nine PT(27%)were treated with radiation therapy(RT)plus concurrent temozolomide(TMZ),22 PT(67%)received TMZ alone,and 2 PT(6%)underwent RT alone.We reported“complete response”in 1 patient(3%),partial response in 9 PT(27%),and stable disease in 15 PT(45%),while 8 PT(25%)had a progressive disease.For all PT,PFS and OS were 19.1 and 30.7 months,respectively.For ECOG PS 0-1 and≥2,PFS was 34.6 months vs.3.4 months(P<0.0001)and OS was 42 months vs.8.9 months(P<0.0001),respectively.Methylated MGMT was associated with longer PFS(41.6 months vs.8.9 months,P=0.05)and OS(52.7 months vs.14.6 months,P=0.009);PFS for IDH1 mutation and IDH wild-type was 52.7 months vs.8.9 months(P=0.006)and OS was 52.7 months vs.41.7 months(P=0.02),respectively.No significant difference was detected as regards treatments.With regard to histological subtype,OS was 42.0 months vs.52.7 months(P=0.8)and PFS was 41.6 months vs.28.6 months(P=0.7)for astrocytoma vs.oligodendroglioma,respectively.PT with treatment response showed a longer OS.PT receiving second-line treatment had a longer OS of 30.7 months vs.6.5 months(P=0.04).Conclusion:ECOG PS,MGMT methylation,and IDH1 mutational status seem to have an important prognostic significance,while the type of treatment does not seem to affect survival.Treatment response could be a surrogate marker for survival.展开更多
文摘Aim:Gliomatosis cerebri(GC)is defined as a rare pattern of growth of diffuse gliomas involving three or more cerebral lobes.Given its rarity,it is difficult to define prognostic factors and standard of treatment.We retrospectively analyzed patients(PT)with GC from a single institution with the aim of identifying the main prognostic factors and to assess optimal management.Methods:Medical records were reviewed of patients≥18 years with a histological and/or radiological diagnosis of GC(with no contrast enhancement)occurring between 2006 and 2017.Median progression free survival(PFS)and overall survival(OS)were calculated by the Kaplan-Meier method.Results:We analyzed 33 PT,22 males and 11 females;Eastern Cooperative Oncology Group(ECOG)performance status(PS)was 0-1 in 21 of the patients.Twenty-two PT underwent biopsy:16 were astrocytomas and 6 oligodendrogliomas.O6-methylguanin-DNA-methyltransferase(MGMT)was detected in 14 cases,and it was methylated in eight cases.Isocitrate dehydrogenase 1(IDH1)was analyzed in 16 PT,and it had mutated in 10 of them.Nine PT(27%)were treated with radiation therapy(RT)plus concurrent temozolomide(TMZ),22 PT(67%)received TMZ alone,and 2 PT(6%)underwent RT alone.We reported“complete response”in 1 patient(3%),partial response in 9 PT(27%),and stable disease in 15 PT(45%),while 8 PT(25%)had a progressive disease.For all PT,PFS and OS were 19.1 and 30.7 months,respectively.For ECOG PS 0-1 and≥2,PFS was 34.6 months vs.3.4 months(P<0.0001)and OS was 42 months vs.8.9 months(P<0.0001),respectively.Methylated MGMT was associated with longer PFS(41.6 months vs.8.9 months,P=0.05)and OS(52.7 months vs.14.6 months,P=0.009);PFS for IDH1 mutation and IDH wild-type was 52.7 months vs.8.9 months(P=0.006)and OS was 52.7 months vs.41.7 months(P=0.02),respectively.No significant difference was detected as regards treatments.With regard to histological subtype,OS was 42.0 months vs.52.7 months(P=0.8)and PFS was 41.6 months vs.28.6 months(P=0.7)for astrocytoma vs.oligodendroglioma,respectively.PT with treatment response showed a longer OS.PT receiving second-line treatment had a longer OS of 30.7 months vs.6.5 months(P=0.04).Conclusion:ECOG PS,MGMT methylation,and IDH1 mutational status seem to have an important prognostic significance,while the type of treatment does not seem to affect survival.Treatment response could be a surrogate marker for survival.
