Adult patients with histologically confirmed
subependymoma or mixed
subependymoma-
ependymoma surgically treated at a tertiary hospital between 1992 and 2020 were identified. A systematic literature review of the PubMed, Embase, Web of Science, and Google Scholar databases from inception until December 4, 2020, was conducted according to
PRISMA guidelines. Data extracted from both groups included demographics, radiographic features,
tumor characteristics, management, and follow-up variables.
RESULTS: Forty-eight unique patients with
subependymoma were identified by chart review; of these patients, 8 (16.7%) had mixed
subependymoma-
ependymoma tumors. The median age at diagnosis was 49 years (IQR 19.8 years), and 26 patients (54.2%) were male. Forty-two patients (87.5%) had intracranial
subependymomas, and 6 (12.5%) had spinal
tumors. The most common presentation was
headache (n = 20, 41.7%), although a significant number of
tumors were diagnosed incidentally (n = 16, 33.3%). Among the 42 patients with intracranial
tumors, 15 (35.7%) had
hydrocephalus, and the most common surgical strategy was a suboccipital approach with or without C1
laminectomy (n = 26, 61.9%). Gross-total resection (GTR) was achieved in 33 cases (68.7%), and 2 patients underwent
adjuvant radiotherapy. Most patients had no major postsurgical complications (n = 34, 70.8%), and only 1 (2.1%) had recurrence after GTR. Of 2036 reports initially identified in the systematic review, 39 were eligible for inclusion, comprising 477 patients. Of 462 patients for whom
tumor location was reported, 406 (87.9%) were intracranial, with the lateral ventricle as the most common location (n = 214, 46.3%). Spinal
subependymomas occurred in 53 patients (11.5%), with 3 cases (0.6%) in multiple locations. Similar to the case series at the authors' institution,
headache was the most common presenting symptom (n = 231, 54.0%) among the 428 patients whose presentation was reported. Twenty-seven patients (6.3%) were diagnosed incidentally, and 36 cases (8.4%) were found at autopsy. Extent of resection was reported for 350 patients, and GTR was achieved in 250 (71.4%). Fifteen of 337 patients (4.5%) had recurrence or progression.
CONCLUSIONS: