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儿童中枢神经细胞瘤:临床病理特征及长期手术结果

Central neurocytomas in children: Clinicopathologic features and long-term surgical outcomes.

作者信息

Zhang Liang, Fang Sheng, Li Xueji

机构信息

National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

Department of Neurosurgery, The Third People's Hospital of Chengdu, Chengdu, China.

出版信息

Childs Nerv Syst. 2023 Jan;39(1):141-150. doi: 10.1007/s00381-022-05663-9. Epub 2022 Sep 12.

Abstract

PURPOSE

Pediatric central neurocytomas (PCNs) is an extremely rare entity, and very few reports have exclusively focused on the clinicopathologic features and surgical outcomes of PCNs.

METHODS

This study was undertaken to elucidate the clinical and pathological characteristics and long-term surgical outcomes of PCNs.

RESULTS

Fourteen consecutive patients with pathologically verified PCNs were identified, including 9 girls and 5 boys, with a mean age of 15.2 ± 3.9 years (range 3-18 years). Children tended to present with symptoms of increased intracranial pressure (ICP) (p = 0.003), an acute clinical course (p = 0.044), and a shorter duration of symptoms (p = 0.019) than their adult counterparts. Surgery was performed under the guidance of a neuronavigation system and intraoperative ultrasound; gross total resection (GTR) was achieved in 12 patients (85.7%), and subtotal resection (STR) was achieved in two patients (14.3%). One child received adjuvant radiotherapy (RT) owing to the atypical features of the lesion. During a mean follow-up of 90.2 ± 37.0 months (range 42-156 months), one patient (7.1%) with atypical features experienced lesion relapse 2 years after surgery and received RT, and the symptoms of all children were relieved.

CONCLUSION

Surgery can benefit children with CNs and ensure relatively long-term progression-free survival. RT can be administered to patients with residual or relapsed lesions.

摘要

目的

儿童中枢神经细胞瘤(PCNs)极为罕见,仅有极少报道专门聚焦于PCNs的临床病理特征及手术结果。

方法

本研究旨在阐明PCNs的临床和病理特征以及长期手术结果。

结果

共纳入14例经病理证实的PCNs患者,其中女孩9例,男孩5例,平均年龄15.2±3.9岁(范围3 - 18岁)。与成年患者相比,儿童患者更易出现颅内压升高(ICP)症状(p = 0.003)、临床病程急性(p = 0.044)且症状持续时间较短(p = 0.019)。手术在神经导航系统和术中超声引导下进行;12例患者(85.7%)实现了全切除(GTR),2例患者(14.3%)实现了次全切除(STR)。1例儿童因病变具有非典型特征接受了辅助放疗(RT)。在平均90.2±37.0个月(范围42 - 156个月)的随访期间,1例具有非典型特征的患者(7.1%)术后2年出现病变复发并接受了RT,所有儿童的症状均得到缓解。

结论

手术可使患有CNs的儿童受益,并确保相对长期的无进展生存期。对于有残留或复发病变的患者可给予RT。

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