Rare central nervous system tumors in adults: a population-based study of ependymomas, pilocytic astrocytomas, medulloblastomas, and intracranial germ cell tumors

• Published in: Neuro-oncology advances Vol. 4; no. 1; p. vdac062
• Main Authors: Ho, Vincent K Y, Gijtenbeek, Anja (J) M M, Wagemakers, Michiel, Taal, Walter, van Linde, Myra E, Swaak-Kragten, Annemarie T, Kurt, Erkan, van der Weide, Hiske L, Wesseling, Pieter, de Vos, Filip Y, Bromberg, Jacoline E C
• Format: Journal Article
• Language: English
• Published: US Oxford University Press 01-01-2022
• Subjects: Basic and Translational Investigations; ependymoma; intracranial germ cell tumor; epidemiology; medulloblastoma; pilocytic astrocytoma
• ISSN: 2632-2498; 2632-2498
• DOI: 10.1093/noajnl/vdac062

Abstract
Background
Ependymomas, pilocytic astrocytomas, medulloblastomas, and intracranial germ cell tumors occur relative frequently in children, but are rare central nervous system (CNS) tumors in adults. In this population-based survey, we established incidence, treatment, and survival patterns for these tumors diagnosed in adult patients (≥18 years) over a 30-year period (1989–2018).
Methods
Data on 1384 ependymomas, 454 pilocytic astrocytomas, 205 medulloblastomas, and 112 intracranial germ cell tumors were obtained from the Netherlands Cancer Registry (NCR) on the basis of a histopathological diagnosis. For each tumor type, age-standardized incidence rates and estimated annual percentage change were calculated. Trends in incidence and main treatment modalities were reported per 5-year periods. Overall survival was calculated using the Kaplan–Meier method, and relative survival rates were estimated using the Pohar-Perme estimator.
Results
Incidence and survival rates remained generally stable for pilocytic astrocytomas, medulloblastomas, and germ cell tumors. Increasing incidence was observed for spinal ependymomas, mostly for myxopapillary ependymomas, and survival improved over time for grade II ependymomas (P < .01). Treatment patterns varied over time with shifting roles for surgery in ependymomas and for chemotherapy and radiation in medulloblastomas and germinomas.
Conclusions
The study provides baseline information for highly needed national and international standard treatment protocols, and thus for further improving patient outcomes in these rare CNS tumors.