Introduction: Adult intracranial ependymomas are rare lesions. Most large single-center studies
remain limited with case numbers and follow-up. We sought to look at a 15-year experience
from our academic institution.
Materials and Methods: We queried the pathology department at our tertiary-care center for specimens positive
for ependymoma from 2009 to 2024 (using natural language processing). We then reviewed
charts to exclude extracranial locations and pathology specimens that were later changed
to nonependymoma lesions once final genomics resulted based on 2022 WHO guidelines.
Once we confirmed we had only intracranial lesions that were positive for ependymoma,
we collected demographics, presenting symptoms, radiographic information, surgical
considerations, and postoperative outcomes.
Results: Over 15 years, 18 pathology-confirmed intracranial ependymomas were resected at our
institution. Of note, 22 patients were identified in our database though four had
to be removed due to final genomic analysis ruling out ependymoma. Median age at time
of surgery was 56 years, and 10 patients (55.6%) were female. Thirteen tumors (72.2%)
were infratentorial, 5 (27.8%) were supratentorial; six cases were entirely extraventricular
(33.4%); 5 (27.8%) had an associated cystic component. Median infratentorial tumor
size was 5.91 cm3 (interquartile range [IQR]: 4.21–18.6), median supratentorial tumor size was 25.7
cm3 (IQR: 4.56–126; p = 0.308). Median duration of symptoms preoperatively was two months. Three patients
(16.7%) had prior resection of their ependymomas. On presentation, seven patients
(38.9%) were diagnosed with hydrocephalus, seven patients (38.9%) had cranial neuropathies
(most commonly VI (N = 3) and VIII (N = 3), eleven patients complained of headache (61.1%), seven (38.9%) of nausea/vomiting,
five (27.8%) of vertigo, ten patients (55.6%) of symptoms attributed to cerebellar
dysfunction, and five patients (27.8%) had signs of weakness on physical exam. According
to operative reports, gross total resection was achieved in ten patients (55.6%) and
near total in four (22.2%). Pathology confirmed 14 cases (77.8%) were WHO grade 2
and 3 cases (16.7%) as grade 3. Three patients (16.7%) were underwent adjuvant chemotherapy,
eleven patients (61.1%) radiotherapy. Median length of follow-up was 21 months. Three
patients (16.7%) died during follow-up. In patients with follow-up, progression free
survival was a median of 42 months and overall survival was 5.5 years. At follow-up,
four patients (22.2%) had new bulbar symptoms, 60% of patients had improvement of
weakness, 90% had improvement of cerebellar symptoms, 80% had improvement of vertigo.
Conclusion: Genomic analysis is essential to proper identification of ependymomas in resected
adult tumors where initial pathology is suggestive of diagnosis. Resection of ependymomas
despite location and size is safe though can cause a high rate of postoperative bulbar
symptoms despite significant improvement in other symptoms. Progression free survival
and overall survival is reasonable with the use of adjunctive therapy when recommended.
