An Overview of Intracranial Ependymomas in Adults

Abstract

Ependymomas are rare primary central nervous system tumors. They can form anywhere along the neuraxis, but in adults, these tumors predominantly occur in the spine and less frequently intracranially. Ependymal tumors represent a heterogenous group of gliomas, and the WHO 2016 classification is based essentially on a grading system, with ependymomas classified as grade I, II (classic), or III (anaplastic). In adults, surgery is the primary initial treatment, while radiotherapy is employed as an adjuvant treatment in some cases of grade II and in all cases of anaplastic ependymoma; chemotherapy is reserved for recurrent cases. In recent years, important and interesting advances in the molecular characterization of ependymomas have been made, allowing for the identification of nine molecular subgroups of ependymal tumors and moving toward subgroup-specific patients with improved risk stratification for treatment-decisions and future prospective trials. New targeted agents or immunotherapies for ependymoma patients are being explored for recurrent disease. This review summarizes recent molecular advances in the diagnosis and treatment of intracranial ependymomas including surgery, radiation therapy and systemic therapies.

Intracranial ependymoma in an adult patient with multiple primary malignancies

“…This is especially so when tumours encase the cranial nerves, brainstem vasculature or ventricles, as seen in this patient. This challenge is more frequent in infratentorial ependymomas (such as in the case of our patient) as compared with supratentorial ones 2. Second-look surgery is recommended following incomplete resection to improve progression-free survival and overall survival rate 3…”

“…This is particularly so in patients with grade III ependymomas and patients with incompletely resected low-grade ependymomas 3. The evidence for grade II ependymomas is debatable; due to the low incidence of adult ependymomas, there are no prospective randomised trials to validate the benefit of radiotherapy in this patient group and the suggestion of an improvement in progress-free survival is based on retrospective data 2. Furthermore, the optimal dose and radiation field are not standardised 3…”

“…They occur more frequently in children than adults, constituting 1.9% of all CNS tumours in adult patients as compared with 5.2% in children 1. Among adults, ependymomas predominantly arise in the spine and less commonly intracranially 2. Ependymomas are classified into grades I, II and III based on their degree of anaplasia to predict clinical prognosis 3.…”

Karakteristik Klinikopatologik dan Luaran Klinik Pasien Ependimoma Di Rsup Dr. Kariadi Semarang

“…14 Ependimoma intrakranial pada orang dewasa disebutkan terjadi sekitar 1-3% kasus pada rentang usia 30-50 tahun dan cenderung tumbuh pada lokasi spinal (23.3% berlokasi di cord, spinal meninges, dan cauda equina). 10,15 Hasil analisis penelitian ini berdasarkan uji beda yang dilakukan, ditemukan perbedaan yang bermakna antara usia kasus terdiagnosis ependimoma dengan luaran klinik pasien, artinya usia saat terdiagnosis tumor ependimoma sangat mempengaruhi luaran klinik dari pasien. Hal ini sesuai dengan pernyataan sebelumnya yang menerangkan bahwa semakin muda usia pasien akan memperlihatkan luaran klinik yang buruk dibandingkan usia lebih dewasa yang cenderung lebih baik, karena lokasi tumbuhnya tumor pada anak paling sering intrakranial dan dewasa sering di SP, yang pada saat pembedahan tumor akan lebih mudah dilakukan reseksi dibandingkan tumor intrakranial.…”