Autran Didier, Barrie Maryline, Matta Mona, Monserrat Colette, Campello Chantal, Petrirena Gregorio, Boucard Celine, Padovani Laetitia, Loundou Anderson, Appay Romain, Graillon Thomas, Dufour Henry, Figarella-Branger Dominique, Chinot Olivier, Tabouret Emeline
Department of Neuro-Oncology, AP-HM, CHU Timone, Marseille, France
Department of Neuro-Oncology, AP-HM, CHU Timone, Marseille, France.
Anticancer Res. 2019 Feb;39(2):1035-1041. doi: 10.21873/anticanres.13210.
BACKGROUND/AIM: Secondary leptomeningeal gliomatosis (LG) is a rare and severe progression pattern of glioma. Our objective was to evaluate the characteristics and outcome of patients with LG.
We retrospectively reviewed 31 patients diagnosed with secondary LG. At the time of LG diagnosis, the median age of patients was 45 years. The histological grade was IV in 20 patients and II to III in 11 patients. As a first-line of therapy for LG, 22 patients received an oncological treatment: i) BCNU-temozolomide (TMZ) (n=15), ii) other type of chemotherapy (n=7), and iii) no treatment (supportive care) (n=9).
Following LG diagnosis, the median progression-free survival (PFS) and overall survival (OS) were 1.8 months [95% confidence interval (CI)=0.9-2.7] and 2.1 months (95%CI=1.3-3), respectively. The univariate analyses showed an improved OS with age of less than 45 years (p<0.001), a prolonged interval from the initial glioma diagnosis (IGD) to LG diagnosis (p=0.003), BCNU-TMZ as the preferred first-line treatment for LG out of the three options (p=0.008), and Karnofsky performance status (KPS) ≥70 (p=0.012). Prolonged interval from IGD to LG diagnosis (HR=5.839) and BCNU-TMZ as the chosen first-line treatment for LG (HR=6.635) remained significant in the multivariate analyses as well. Among the 22 treated patients, the median OS was significantly higher (p=0.008) with the BCNU-TMZ treatment (5.7 months; 95%CI=4.2-7.1), compared to other types of treatment offered (2 months; 95%CI=1.1-2.9).
The time interval from the IGD to the LG diagnosis is a potential prognostic factor for LG. BCNU-TMZ may be a therapeutic option in the present setting.
背景/目的：继发性软脑膜胶质瘤病（LG）是一种罕见且严重的胶质瘤进展模式。我们的目的是评估LG患者的特征和预后。
我们回顾性分析了31例诊断为继发性LG的患者。在LG诊断时，患者的中位年龄为45岁。组织学分级为IV级的患者有20例，II至III级的患者有11例。作为LG的一线治疗，22例患者接受了肿瘤治疗：i）卡莫司汀-替莫唑胺（TMZ）（n = 15），ii）其他类型的化疗（n = 7），以及iii）未治疗（支持治疗）（n = 9）。
LG诊断后，中位无进展生存期（PFS）和总生存期（OS）分别为1.8个月[95%置信区间（CI）= 0.9 - 2.7]和2.1个月（95%CI = 1.3 - 3）。单因素分析显示，年龄小于45岁（p < 0.001）、从初始胶质瘤诊断（IGD）到LG诊断的间隔时间延长（p = 0.003）、在三种选择中BCNU - TMZ作为LG的首选一线治疗（p = 0.008）以及卡诺夫斯基功能状态（KPS）≥70（p = 0.012）时，OS有所改善。在多因素分析中，从IGD到LG诊断的间隔时间延长（HR = 5.839）以及BCNU - TMZ作为LG的首选一线治疗（HR = 6.635）仍然具有显著性。在22例接受治疗的患者中，与其他类型的治疗（2个月；95%CI = 1.1 - 2.9）相比，BCNU - TMZ治疗组的中位OS显著更高（p = 0.008）（5.7个月；95%CI = 4.2 - 7.1）。
从IGD到LG诊断的时间间隔是LG的一个潜在预后因素。在当前情况下，BCNU - TMZ可能是一种治疗选择。
