Abstract

BACKGROUND: Secondary leptomeningeal gliomatosis (LMG) is a rare and severe progression pattern of glioma. Prognostic factors and therapeutic management of this pattern remains unclear. Our objective was to evaluate characteristics and outcome of LMG patients (pts). METHODS: We retrospectively reviewed pts diagnosed with secondary LMG referred to our institution between 2004 and 2013. Diagnosis was obtained by clinical, imaging and cerebrospinal fluid (CSF) data. RESULTS: The identification and diagnosis of 31 pts with LMG was based on CSF cytology (n = 12), on MRI data (n = 16) with hyperproteinarrachia (>1.2 gr/l) (n = 9) or without (n = 7), or on meningism with hyperproteinarrachia (n = 3). At LMG diagnosis (LMGD), median age was 45 years and Karnofsky Performance Status (KPS) was <70 in 71% of pts; histological grade was IV in 20 pts and II-III in 11 pts. Median interval from initial glioma diagnosis (IGD) to LMGD was 13.5 months (glioblastoma: 8.1; grades II-III: 28.9). As first line therapy for LMG, 22 pts received an oncological treatment: BCNU-temozolomide (TMZ), n = 15; other chemotherapy, n = 7; craniospinal radiotherapy: n = 0; no further trt, n = 9. From LMGD, median PFS and median OS were 1.8 months (95% confidence interval [CI]: 0.9-2.7) and 2.1 months (CI95%: 1.3-3) respectively. Clinical improvement was observed in 6/22 treated patients, of which 3 showed concomitant imaging improvement; 13/22 pts exhibit a rapid decline. Univariate analyses showed an improved OS with age <45 years old (p < 0.001), prolonged interval from IGD to LMGD (p = 0.003) and KPS ≥70 (p = 0.012). Remained significant in the multivariate analysis: age (HR: 4.533) and interval since IGD (HR: 4.803). Among the 22 treated patients, median OS was significantly higher with BCNU-TMZ trt: 5.7 months (CI95%: 4.2-7.1) vs 2 months (CI95%: 1.1-2.9) for other trt (p = 0.008). Initial proteinorrachia and its changes during LMG treatment were not correlated to response and did not influence outcome. CONCLUSION: We identify age and time interval from IGD to LMGD as potential prognostic factors for LMGD. BCNU-TMZ combination could be an interesting therapeutic option for these patients.

Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call