Clinical Features and Prognostic Risk Factors of Choroid Plexus Tumors in Children

Chin Med J (Engl). 2018 Dec 20;131(24):2938-2946. doi: 10.4103/0366-6999.247195.


Background: Decision-making concerning the treatment of choroid plexus tumor (CPT) in pediatric patients remains a topic of considerable debate. The aim of this work was to describe clinical features and prognostic risk factors of CPT in the pediatric population and to provide theoretical opinions regarding clinical decisions for CPT.

Methods: The data of 96 patients with CPT and younger than 14 years were retrospectively analyzed. Clinical characteristics such as pathological type of CPTs, rate and severity of hydrocephalus, treatment and outcome, and recurrence were investigated. For categorical variables, the Pearson's Chi-square test was performed. The Mann-Whitney U-test was used for comparisons between nonnormally distributed parameters. Log-rank test was used for progression-free survival (PFS).

Results: The study included 70 choroid plexus papilloma (CPP) cases, 17 atypical choroid plexus papilloma (aCPP) cases, and 9 choroid plexus carcinoma (CPC) cases. Compared with patients with CPP or aCPP, patients with CPC had a shorter disease course (median: CPP, 4 months; aCPP, 2 months; CPC, 1 month; H: 23.5, P < 0.001), higher rate of acute hydrocephalus (CPP, 27.1%; aCPP, 52.9%; CPC, 77.8%; χ2 = 10.9, P < 0.05), and lower incidence of cure rate (CPP, 85.7%; aCPP, 70.5%; CPC, 33.3%; χ2 = 13.5, P < 0.05). The severity of hydrocephalus with tumor in the lateral or third ventricle was significantly higher than that with tumors in the fourth ventricle (severe hydrocephalus: lateral ventricle, 51.7%; third ventricle, 47.0%; fourth ventricle, 11.1%; χ2 = 26.0, P < 0.001). Patients with gross total surgical resection had no better PFS than those with partial resection because of the use of adjuvant therapy in the latter (χ2 = 4.0, P > 0.05). Patients with CPC experienced shorter time for recurrence than those with CPP or aCPP (χ2 = 40.1, P < 0.0001).

Conclusions: Our results indicated that CPP in the fourth ventricle could trigger serious clinical symptoms at an early stage, requiring early intervention. Adjuvant treatment might be necessary for patients with partially resected CPP, aCPP, and CPC to achieve a favorable outcome.

小儿脉络丛肿瘤的临床特征及预后危险因素的相关研究 摘要 背景:目前针对小儿脉络丛肿瘤的诊疗决策仍具争议。本研究通过探讨小儿脉络丛肿瘤的临床资料,为小儿脉络丛肿瘤的诊疗决策提供理论基础。 方法:对本中心96例小于14周岁的脉络丛乳头状肿瘤患者的临床资料进行回顾性分析。本研究纳入包括脉络丛乳头状瘤患者的病理分型、脑积水的发病率以及严重程度、治疗方式、预后以及复发情况在内的临床资料进行统计分析。 皮尔逊卡方检验用于分类数据。曼-惠特尼秩和检验用于非正态分布的数据。时序检验用于无进展生存曲线。 结果:本研究共入组70例脉络丛乳头状瘤患者、17例非典型脉络丛乳头状瘤患者和9例脉络丛乳头状癌患者。脉络丛乳头状癌患者较其他几种类型的患者病程更短(中位数, CPP: 4 月; aCPP: 2 月; CPC: 1 月, H: 23.5, P< 0.001),急性脑积水发病率更高(CPP: 27.1%; aCPP: 52.9%; CPC:77.8%, χ2=10.9, P<0.05)以及较低的治愈率(CPP: 85.7%; aCPP: 70.5%; CPC: 33.3%, χ2=13.5, P<0.05)。肿瘤位于侧脑室以及三脑室的患者脑积水的严重程度明显高于肿瘤位于四脑室的患者(重度脑积水:侧脑室,51.7%;三脑室,47.0%;四脑室,11.1%,χ2=26.0,P<0.05)。由于术后辅助放化疗的干预,肿瘤大部切除的患者其预后较肿瘤全切的患者没有明显差异(χ2=4.0,P>0.05)。本组研究还发现在脉络丛肿瘤患者中,病理类型是影像预后的一个危险因素(χ2=40.1,P<0.0001)。 结论本研究的结果显示位于四脑室的脉络丛乳头状肿瘤患者在早期即可出现明显的临床症状,需要早期干预。对于次全切除的脉络丛乳头状肿瘤患者、非典型脉络丛乳头状瘤患者和脉络丛乳头状癌患者,术后辅助治疗可以获得理想的预后。.

Keywords: Atypical Choroid Plexus Papilloma; Choroid Plexus Carcinoma; Choroid Plexus Papilloma; Pediatric Patients.

MeSH terms

  • Child
  • Child, Preschool
  • Choroid Plexus Neoplasms / mortality*
  • Choroid Plexus Neoplasms / pathology
  • Choroid Plexus Neoplasms / surgery
  • Female
  • Humans
  • Hydrocephalus / etiology
  • Infant
  • Male
  • Neoplasm Metastasis
  • Neoplasm Recurrence, Local
  • Prognosis
  • Risk Factors