Surgical management and clinical outcomes of cerebellar liponeurocytomas-a report of 7 cases and a pooled analysis of individual patient data

Abstract

Objective Cerebellar liponeurocytomas (CLPNs) are very rare. Limited studies described this disease and their treatment protocol remain unclear. To better understand the disease, we review the clinical features and outcomes, and propose a treatment protocol based on previously reported cases and cases from our institute. Methods The clinical data were obtained from 7 patients with pathologically confirmed CLPNs, who accepted surgical treatment in our institute between November 2011 and June 2021. We also reviewed the literature and 75 patients with CLPNs were identified between September 1993 and June 2021. Risk factors for Progression-free survival (PFS) were evaluated in the pooled cohort. Results The authors’ cohort included 4 males and 3 females with a mean age of 43.9±14.5 (range: 29-64 years). 3 cases are located in lateral ventricle and 4 cases are located in cerebellum. All 7 cases achieved gross total resection (GTR) and radiotherapy was administered to 2 cases. After a mean follow-up of 44.9±44.4 months, all patients remained well with no recurrence or death. For the reported 75 patients, there are 35 males and 40 females with a mean age of 46.2±13.6 years (range: 6-77 years). Biopsy, gross total resection (GTR) and non-GTR were achieved in 1 (1.3%), 50 (66.7%), and 24 (32%) patients, respectively. Radiotherapy was administered to 16 cases and chemotherapy was administered to only 1 case. After a mean follow-up of 47.5±51.5 months, 3 patients died and tumor recurrence occurred in 17 patients. Multivariate Cox analysis revealed that non-GTR predicted a poor PFS (p=0.030). Kaplan-Meier analysis showed that GTR was significantly associated with better PFS (p=0.0084). PFS rates at 1, 5, 10 years were 92.7%, 78.0%, 23.8% respectively. Conclusions Cerebellar liponeurocytomas (CLPNs) are very rare brain tumors. Although they have favorable clinical prognosis, the recurrence is relatively high. GTR should be the first choice and close follow-up is necessary. Postoperative radiotherapy could not improve PFS in this study. A larger cohort is needed to verify our findings.