Elsevier

World Neurosurgery

Volume 146, February 2021, Pages e799-e810
World Neurosurgery

Original Article
Pineal Region Glioblastomas: Clinical Characteristics, Treatment, and Survival Outcome

https://doi.org/10.1016/j.wneu.2020.11.016Get rights and content

Objective

Given the rarity of patients with pineal glioblastoma (GBM), clinical characteristics, treatment, and prognostic factors are not well characterized. This study aimed to investigate these characteristics and identify the prognostic factors of overall survival (OS).

Methods

A retrospective analysis of newly diagnosed patients with pineal GBM, including our 3 cases and an additional 44 cases from published articles, was conducted. Survival analysis was performed by Kaplan-Meier analysis and Cox regression analysis was used to determine the prognostic factors.

Results

A total of 47 patients (28 males and 19 females) were enrolled, with a median age of 46 years (range, 5–74 years). Forty-four patients (90.9%) had preoperative obstructive hydrocephalus. Among 38 patients, 21 (55.3%) had distal leptomeningeal dissemination. Forty-five patients (95.7%) had resection/biopsy, 6 of whom had gross total resection, 22 had subtotal resection, 7 had partial resection, and 10 had biopsy. Adjuvant therapy included radiotherapy in 36 patients and chemotherapy in 27 patients. The median OS was 10.0 months. The 6-month, 1-year, and 2-year survival was 68.0%, 42.6%, and 17.0%, respectively. Cox regression analysis showed that patients receiving biopsy (P = 0.042) or chemotherapy (P = 0.029) had the better OS and these were regarded as independent prognostic factors. Further survival analysis showed that chemoradiotherapy had better survival benefit than other regimens.

Conclusions

In this study, we summarized the characteristics of patients with pineal GBM and showed the correlation between clinical characteristics and prognosis. This study may give readers a deep understanding of these rare GBMs and provide some references for future management.

Introduction

Pineal region tumors account for about 0.4%–1% of all intracranial tumors in adults and contain a wide variety of histologic types, generally comprising germ cell, pineal parenchymal, and extrapineal tumors arising from the surrounding parenchyma.1, 2, 3, 4 Glioma arising from the surrounding glial stroma is a rare subtype of pineal region tumors and malignant gliomas or glioblastomas (GBMs) located in this region are extremely rare.4, 5, 6, 7 To our knowledge, pineal region gliomas are either included in larger series along with other tumors or reported as case reports/series.6, 7, 8, 9, 10, 11 Few cases with pineal region GBM (also called pineal GBM) have been reported in the English-language literature. Little is known about the clinical characteristics, treatment, and prognosis of this rare entity. In this study, we report 3 patients with pineal GBM and thoroughly review the English-language literature, to summarize the clinical characteristics and treatment strategy in the patients with pineal GBM and determine the prognostic factors of overall survival (OS).

Section snippets

Source of Patients

We searched the glioma database in our institution and identified 3 patients with newly diagnosed pineal GBM between 2016 and 2019. We also thoroughly searched the English-language literature about pineal GBM from PubMed and Web of Science and identified 44 patients with pineal GBM from 28 articles published between 1954 and 2020.6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33 The key terms used for the standard retrieval strategy were

Patient Demographics and Clinical Characteristics

A total of 47 patients (28 males and 19 females) with pineal GBM were included in this study. The flow diagram of case selection and inclusion according to the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines are shown in Figure 1. The mean age of all patients was 41.5 ± 18.9 years, and the median age was 46 years (range, 5–74 years). The duration of symptoms ranged from 1 day to 108 months, with a median duration of 1 month. In patients for whom data of

Discussion

Pineal GBMs are extremely uncommon intracranial tumors.1, 2, 3, 4,10 A previous review showed that pineal GBMs accounted for approximately 20% of pineal gliomas.4 Usually, pineal GBMs are reported in case reports/series. To the best of our knowledge, there is a lack of integrative study focusing on the clinical characteristics, treatment, and prognosis of exclusively pineal GBMs. This study included 47 patients with pineal GBM and the findings showed that these patients had poor survival

Conclusions

In this study, we summarized the characteristics of patients with pineal GBM based on individual data and showed the correlation between clinical characteristics and prognosis. This study may give readers a deep understanding of these rare tumors and provide some references for future management. However, these conclusions should be interpreted cautiously and further studied.

CRediT authorship contribution statement

Xiaodong Niu: Conceptualization, Methodology, Software, Writing - original draft. Chenghong Wang: Data curation, Writing - original draft. Xingwang Zhou: Data curation, Writing - original draft. Yuan Yang: Methodology, Visualization, Supervision. Yanhui Liu: Validation, Supervision. Yuekang Zhang: Writing - review & editing, Supervision. Qing Mao: Writing - review & editing, Supervision.

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    Conflict of interest statement: This study was supported by the Sichuan Provincial Science and Technology Program (number 2018SZ0143), the Innovation and Sparkle Project of Sichuan University (number 2082604401004/060) and the Technology Innovation Research and Development Project of Chengdu (number 2019-YF05-00392-SN).

    Xiaodong Niu, Chenghong Wang, and Xingwang Zhou are co–first authors.

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