Surgical management and long-term outcome of intracranial subependymoma

Abstract

BackgroundIntracranial subependymomas account for 0.2–0.7% of central nervous system tumours and are classified as World Health Organization (WHO) grade 1 tumours. They are typically located within the ventricular system and are detected incidentally or with symptoms of hydrocephalus. Due to paucity of studies exploring this tumour type, the objective was to determine the medium- to long-term outcome of intracranial subependymoma treated by surgical resection.MethodsRetrospective case note review of adults with intracranial WHO grade 1 subependymoma diagnosed between 1990 and 2015 at the Walton Centre NHS Foundation Trust was undertaken. Tumour location, extent of resection (defined as gross total resection (GTR), sub-total resection (STR) or biopsy) and the WHO performance status at presentation and through follow-up were recorded.ResultsThirteen patients (7 males; 6 females) with a mean age of 47.6 years (range 33–58 years) and a median follow-up of 46 months (range 25–220 months) were studied. Eight patients had symptomatic tumours (headache, visual disturbance); five had incidental finding. Tumours were most commonly located in the fourth ventricle (n = 8). The performance status scores at diagnosis were 0 (n = 8) and 1 (n = 5). The early post-operative performance status scores at 6 months were 0 (n = 5) and 1 (n = 8) and at last follow-up were 0 (n = 11) and 1 (n = 2). There was no evidence of tumour re-growth following GTR or STR. The commonest complication was hydrocephalus (n = 3).ConclusionSubependymoma are indolent tumours. No patients exhibited a worsening of performance status at medium- to long-term follow-up and there were no tumour recurrence suggesting a shorter follow-up time may be sufficient. Surgical resection is indicated for symptomatic tumours or those without a clear imaging diagnosis. Incidental intraventricular subependymoma can be managed conservatively through MRI surveillance.

Intraventricular neuroepithelial tumors: Surgical outcome, technical considerations and review of literature

“…Therefore a "watch and wait" strategy with regular MRI follow-ups can lead to undertreatment in case of more aggressive entities mistaken for a subependymoma. We report on 21 patients, representing the second largest "surgical" series published to date (table 5) (3,(56)(57)(58)(59)(60)(61)(62)(63)(64). As radiation or systemic treatment do not apply for subependymomas, surgery remains the only viable option in this entity.…”

“…The surgical strategy focusses on maximal but safe resection, resulting in permanent absence of the tumor. In the majority of reports, gross total resection could be achieved in >70% of patients, with low rates of mortality and morbidity after surgery (3,(56)(57)(58)(59)(60)(61)(62)(63)(64). In our cohort, we were able to achieve gross total resection in all cases (21/21) with a surgery-related mortality rate of 4,8%.…”

Intraventricular neuroepithelial tumors: Surgical outcome, technical considerations and review of literature

“…Most published reports on subependymomas represent smaller and retrospective cohorts (2,(58)(59)(60)(61)(62)(63)(64)(65)(66). The largest report of Elisabeth Rushing et al comprised 83 cases, but focused on histopathological ndings and does not consider surgical aspects (2).…”

“…The surgical strategy focusses on maximal but safe resection, resulting in permanent absence of the tumor. In the majority of reports, gross total resection could be achieved in >70% of patients, with low rates of mortality and morbidity (2,(58)(59)(60)(61)(62)(63)(64)(65)(66). In our cohort, we were able to achieve gross total resection in all cases (21/21) with a surgery-related mortality rate of 4,8%.…”

Intracranial ependymomas: molecular insights and translation to treatment

“…The World Health Organization's (WHO) classification of CNS tumors, categorizes ependymal tumors into five subtypes across three grades . Grade I comprises subependymomas and myxopapillary ependymomas, which are considered benign and rarely transform into grade II or III tumors . However, there are reports of a small number of myxopapillary ependymomas which contain anaplastic features and display malignant behavior .…”