Increased Survival and Symptom Relief Following Palliative Cerebrospinal Fluid Diversion for Neoplastic Leptomeningeal Disease: A Case Series and Systematic Review

Abstract

INTRODUCTION: Leptomeningeal disease (LMD) indicates widespread or advanced cancer and can lead to symptomatic hydrocephalus, for which palliative cerebrospinal fluid (CSF) diversion may be indicated. METHODS: We analyzed survival and symptom relief outcomes from a 10-year period at a single institution of patients with LMD secondary to cancer, symptoms of obstructive hydrocephalus, and documented date of death. We also searched PubMed, Embase, and Web of Science from database inception until 8/20/2022, using search terms including ‘cancer’, ‘hydrocephalus’, and ‘shunt’. Preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines were followed. RESULTS: In our series of 50 patients, 30 (60%) underwent CSF diversion surgery after LMD diagnosis with comparable median age in both surgery (58.4 ± 14.4 years) and non-surgery (57.8 ± 14.5 years) groups. Twenty-three patients (76.7%) achieved symptom relief. The surgery group lived longer after LMD diagnosis than patients in the non-surgery group (6.6 ± 6.0 vs. 1.3 ± 4.3 months, p < 0.001) and had higher likelihood of survival (hazard ratio 2.49, 95% confidence interval 1.37-4.52, p = 0.002). Median survival after surgery was 2.8 ± 3.8 months. Out of 23 articles reporting outcomes for 2895 patients, 995 (34.3%) presented with LMD. Postoperative complication rates were 0-37.7% with no cases of extraneural metastases. Symptom relief rates were 50-100%. Three studies reported median survival after surgery for LMD (2-3.3 months). CONCLUSIONS: A rare entity with dismal prognosis, LMD can present as symptomatic hydrocephalus in patients with cancer. Symptom relief can be achieved with palliative surgery for permanent CSF diversion with relatively low complication rates. Prospective studies are needed to further assess outcomes and needs of this patient population.