Clinical Features, Treatment, and Long-term Outcomes of Central Neurocytoma: A 20-Year Experience at a Single Center

Objective

Central neurocytoma (CN) is a rare central nervous system tumor the optimal management strategy of which remains controversial because of their rarity. Detailed information on CN is necessary to establish optimal management strategies. The purpose of this study was to show the clinical features, treatments, and long-term clinical outcomes of CN.

Methods

A total of 63 patients with CN were surgically treated between 1995 and 2016 at West China Hospital. All pathologically proven CN cases were identified. Epidemiologic characteristics, clinical features, imaging features, functional outcomes, overall survival, and progression-free survival according to multimodal treatments were reviewed retrospectively.

Results

There were 29 males and 34 females, with a median age of 29 years (range, 15–58 years). Thirty-four patients underwent gross total resection (GTR) and 29 patients underwent subtotal resection (STR). The surgical approaches to CNs in this study included a transcortical approach (39 cases) and an interhemispheric transcallosal approach (24 cases). There were no differences in functional outcomes or initial extent of resection according to the surgical approach used. The extent of resection was GTR in 34 patients (54.0%), and STR in 29 patients (46.0%). At the median follow-up of 74 months (range, 6–205 months), the actuarial 5-year and 10-year overall survival were 93.3% and 85.4%. At the last follow-up, 8 patients had experienced tumor progression. The 5-year and 10-year progression-free rate was 73.4% and 57.5%. The initial extent of resection and multimodal treatment was not related to overall survival; however, the actuarial local control rate differed significantly according to the initial extent of resection and multimodal treatment.

Conclusions

CN is a rare type of World Health Organization grade II primary brain tumor with a tendency to recur. Complete resection of CNs with maximal safety remains the primary treatment to minimize local progression. Adjuvant radiotherapy should be considered in patients receiving incomplete resection. The long-term clinical outcomes of CN after multimodal treatment seem to be satisfactory.

Introduction

Central neurocytoma (CN) is a rare central nervous system tumor. The incidence of CN is 0.1%–0.5% among all primary brain tumors.¹ It was first described in 1982 by Hassoun et al.² Depending on the 2016 classification system of the World Health Organization (WHO), CNs are classified as grade II.³ CN often occurred in young adults and were mainly located in deep midline structures near the foramen of Monro.¹ Most CNs are benign, well-differentiated tumors, with favorable prognoses after multimodal therapy. Because of the rarity of this tumor, a clinical randomized controlled trial seems to be impossible. Most clinical research has been based on case reports and retrospective studies of small samples. Although the diagnosis of CN has increased with the accumulation of clinical data, including its clinical, radiologic, and histopathologic characteristics, only <1000 cases of CN have been reported.⁴

Because the numerous aspects of clinical management of CN remain unclear, a thorough investigation of the long-term outcome of CN after multimodal treatments is necessary to establish the optimal therapeutic strategies for these tumors. We collected details of all cases surgically treated and pathologically confirmed as CN at West China Hospital from 1995 to 2016. The clinical features, radiologic characteristics, treatment strategies, and long-term outcomes of patients with CN were reviewed and analyzed retrospectively.