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.
背景：癌症引起的软脑膜疾病（LMD）属于晚期癌症，可导致梗阻性脑积水，此时可能需要姑息性脑脊液（CSF）分流术以缓解症状。本研究调查了成人LMD患者脑积水的手术结果，并对儿童及成人病例进行了系统性综述。 方法：我们分析了10年间患有肿瘤性LMD、梗阻性脑积水且有明确死亡日期的患者结局。同时，我们检索了自建库至2022年8月20日的数据库，使用的检索词包括“癌症”、“脑积水”和“分流术”。研究遵循系统综述和荟萃分析指南的优先报告条目。 结果：在50例患者中，30例（60%）在LMD确诊后接受了CSF分流术，手术组（58.4±14.4岁）与非手术组（57.8±14.5岁）的中位年龄相近。23例患者（76.7%）症状得到缓解。手术组在LMD确诊后的生存期长于非手术组（6.6±6.0个月 vs. 1.3±4.3个月，p<0.001），且生存可能性更高（风险比2.49，95%置信区间1.37–4.52，p=0.002）。术后中位生存期为2.8±3.8个月。从23篇文献中汇总的995例患者中，34.3%表现为LMD。并发症发生率为0–37.7%，未见神经系统外转移。症状缓解率为50–100%。三项研究报告了术后中位生存期（2–3.3个月）。 结论：LMD是一种预后不良的罕见疾病，在癌症患者中可表现为症状性脑积水。姑息性CSF分流术可实现症状缓解并提高生存率，且并发症发生率较低。未来需要前瞻性研究来评估这些患者的结局和需求。
肿瘤(癌症)患者之家