Clinical features and surgical outcomes of intracranial and spinal cord subependymomas

Jordina Rincon-TorroellaDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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Maureen RakovecDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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Adham M KhalafallahDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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Ann LiuDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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Anya BettegowdaDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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Carmen KutDepartment of Radiation Oncology and Molecular Radiation Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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Fausto J. RodriguezDepartment of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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Jon WeingartDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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Mark LucianoDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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Alessandro OliviDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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George I. JalloDepartment of Neurosurgery, Johns Hopkins Medicine, Institute for Brain Protection Sciences, Johns Hopkins All Children’s Hospital, St. Petersburg, Florida; and

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Henry BremDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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Debraj MukherjeeDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;
Johns Hopkins University Bloomberg School of Public Health, Baltimore, Maryland

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Michael LimDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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Chetan BettegowdaDepartment of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland;

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OBJECTIVE

Subependymomas are low-grade ependymal tumors whose clinical characteristics, radiographic features, and postsurgical outcomes are incompletely characterized due to their rarity. The authors present an institutional case series and a systematic literature review to achieve a better understanding of subependymomas.

METHODS

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

The authors’ case series and literature review demonstrate that patients with subependymoma are well managed with resection and generally have a favorable prognosis.

ABBREVIATIONS

EOR = extent of resection; ETV = endoscopic third ventriculostomy; EVD = external ventricular drain; GTR = gross-total resection; KPS = Karnofsky Performance Scale; STR = subtotal resection.
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Illustration from Di Somma et al. (pp 1187–1190). Published with permission from Glia Media | Artist: Martha Headworth, MS.

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