Original ArticlePineal Region Glioblastomas: Clinical Characteristics, Treatment, and Survival Outcome
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.