Atypical clinical manifestations and prognosis analysis of pediatric diffuse intrinsic pontine glioma

Abstract

Objective To analyze the atypical clinical manifestations and clinical prognosis of pediatric diffuse intrinsic pontine glioma (DIPG). Methods A total of 27 patients with complete clinical data of DIPG undergoing clinical treatment at Neurosurgery Department of Beijing Tiantan Hospital, Capital Medical University from November 2015 to November 2017 were retrospectively enrolled into this study. All patients were divided into 2 groups according to the presence of atypical manifestations: atypical clinical manifestation group (17 cases) and the control group (10 cases). Among the 27 cases, 16 patients underwent stereotactic biopsy or open cranial biopsy, and the other 11 patients underwent tumor excision through craniotomy. All the children were followed up clinically or by telephone after operation to study the clinical outcomes. Comparative analysis was conducted on those clinical data. Prognosis was compared between the 2 groups through Kaplan-Meier survival analysis. Results Among the 27 cases, 20 were pathologically identified as high grade gliomas (including 4 cases of diffuse midline glioma, 6 cases of glioblastoma, 6 cases of anaplastic oligoastrocytoma, 4 cases of anaplastic astrocytoma) and 7 as low grade glioma (including 4 cases of oligoastrocytoma and 3 cases of astrocytoma). One child suffered from pneumonia and finally recovered postoperatively. No statistically significant difference (all P>0.05) was identified between 2 groups in the age, gender, clinical manifestation (including cranial nerve palsy, long tract dysfunction and cerebellum signs), clinical imaging features (including lesion enhancement, supratentorial ventricle enlargement and brachium pontis involvement), clinical treatment or pathology. Kaplan-Meier survival analysis showed that patients with atypical clinical manifestation had significantly higher cumulative survival rate than those in the control group (P=0.045). Conclusion Atypical clinical manifestation could be identified in some pediatric DIPG patients, which might be predictive of a better prognosis. Key words: Brainstem tumor; Child; Atypical clinical manifestation