Abstract
BACKGROUND: Leptomeningeal disease (LMD) from cancer indicates advanced cancer and can lead to obstructive hydrocephalus, for which palliative cerebrospinal fluid (CSF) diversion may be indicated to alleviate symptoms. We investigated surgical outcomes for hydrocephalus for adult patients with LMD and conducted a systematic review on pediatric and adult cases. METHODS: We analyzed outcomes from a 10-year period of patients with neoplastic LMD, obstructive hydrocephalus, and documented date of death. We also searched databases from inception until 20 August 2022, using search terms including 'cancer', 'hydrocephalus', and 'shunt'. Preferred reporting items for systematic reviews and meta-analyses guidelines were followed. RESULTS: Among 50 patients, 30 (60%) underwent CSF diversion after LMD diagnosis with comparable median age in both the 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 the non-surgery group (6.6 ± 6.0 vs. 1.3 ± 4.3 months, p < 0.001) and had a 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. From 23 articles, 995 patients (34.3%) presented with LMD. Complication rates were 0-37.7% with no extraneural metastases. Symptom relief rates were 50-100%. Three studies reported median survival after surgery (2-3.3 months). CONCLUSIONS: A rare entity with dismal prognosis, LMD can present as symptomatic hydrocephalus in patients with cancer. Symptom relief and improved survival can be achieved with palliative CSF diversion with low complication rates. Prospective studies are needed to assess the outcomes and needs of these patients.