====== Supratentorial hemispheric ependymoma ====== Supratentorial extraventricular ependymomas are relatively rare. ====Clinical features==== [[Supratentorial ependymoma]]s may produce manifestations of increased intracranial pressure, including headache, nausea, vomiting, and bilateral papilledema. They may also cause focal deficits such as aphasia and motor impairment. Seizures have been reported to occur in one third of the cases. see [[Cerebral myxopapillary ependymoma]]. ===== Outcome ===== Long-term outcomes and prognostic factor for this rare tumor have not been well established. ===== Case series ===== ====2017==== Fifty-five patients with supratentorial extraventricular ependymomas from our hospital were included in this study. Epidemiological characteristics, clinical features, treatment,long-term outcomes, and prognostic factors for PFS and OS were reviewed retrospectively. RESULTS: The patients consisted of 30 males and 25 females with mean age of 30.0 ± 23.6 years (range, 1-74 years). Twenty-nine tumors were located in the right hemisphere, and 26 in the left side. The 2 most common tumor locations were the frontal (n = 19; 35%) and parietal lobe (n = 11; 20%). All patients underwent surgical resection. Gross-total resection (GTR) was achieved in 42 cases (76%) and subtotal resection (STR) was performed in 13 patients (24%). According to the WHO classification system, 38 tumors (69%) were Grade III (anaplastic ependymoma), and 17 (31%) were Grade II (ependymoma). Three-,5-, and 10 year PFS rates were 60%, 49%, and 36%, respectively. Three-,5-, and 10 year OS rates were 79%, 64%, and 49%, respectively. EOR and tumor grade were identified as prognostic factors for PFS and OS on univariate analysis, multivariate analysis, and Kaplan-Meierlog-rank testing. Subtotal resection (STR) predicted a worse PFS (HR = 4.808; 95%, 1.942-11.905; P = .001) and OS (HR = 5.650; 95%, 2.114-15.152; P = .001). WHO Grade III tumors also had worse PFS (HR = 3.922; 95%, 1.429-18.182; P = .012) and OS (HR = 6.329; 95%, 1.328-30.303; P = 0.021). For patients with tumor recurrence, reoperation was significant prognostic factors for OS (HR = 2.091; 95%, 0.939-4.654; p = .000). Age, sex, tumor side, and postoperativeradiotherapy were not prognostic factors for PFS and OS. CONCLUSIONS: Most supratentorial extraventricular ependymomas are WHO grade III tumors. STRandWHO Grade III pathology predicted worse PFS and OS. Gross-total resection remains the optimal treatment for patients with supratentorial extraventricular ependymoma. Reoperation should be considered first in cases of recurrence. The role of postoperative radiotherapy as an adjuvant treatment for supratentorial extraventricular ependymoma needs further investigation ((Wang M, Zhang R, Liu X, Li D, Qiu C, Zhao P, Zuo Y, Zhang P, Wang J, Sun H. Supratentorial extraventricular ependymomas: A retrospective study focused on long-term outcomes and prognostic factors. Clin Neurol Neurosurg. 2017 Dec 13;165:1-6. doi: 10.1016/j.clineuro.2017.12.013. [Epub ahead of print] PubMed PMID: 29253745. )). ====2016==== Hollon et al designed a retrospective study to determine [[progression free survival]] (PFS), [[overall survival]] (OS), and prognostic factors for hemispheric ependymomas. The study population consisted of 8 patients and 101 patients from the literature with disaggregated survival information (n = 109). Patient age, sex, tumor side, tumor location, [[extent of resection]] (EOR), tumor grade, postoperative chemotherapy, radiation, time to recurrence, and survival were recorded. Kaplan-Meier survival analyses and Cox proportional hazard models were completed to determine survival rates and prognostic factors. Anaplastic histology/WHO Grade III tumors were identified in 62% of cases and correlated with older age. Three-, 5-, and 10-year PFS rates were 57%, 51%, and 42%, respectively. Three-, 5-, and 10-year OS rates were 77%, 71%, and 58%, respectively. EOR and tumor grade were identified on both Kaplan-Meier log-rank testing and univariate Cox proportional hazard models as prognostic for PFS and OS. Both EOR and tumor grade remained prognostic on multivariate analysis. Subtotal resection (STR) predicted a worse PFS (hazard ratio [HR] 4.764, p = 0.001) and OS (HR 4.216, p = 0.008). Subgroup survival analysis of patients with STR demonstrated a 5- and 10-year OS of 28% and 0%, respectively. WHO Grade III tumors also had worse PFS (HR 10.2, p = 0.004) and OS (HR 9.1, p = 0.035). Patients with WHO Grade III tumors demonstrated 5- and 10-year OS of 61% and 46%, respectively. Postoperative radiation was not prognostic for PFS or OS. A high incidence of anaplastic histology was found in hemispheric ependymomas and was associated with older age. EOR and tumor grade were prognostic factors for PFS and OS on multivariate analysis. STR or WHO Grade III pathology, or both, predicted worse overall prognosis in patients with hemispheric ependymoma ((Hollon T, Nguyen V, Smith BW, Lewis S, Junck L, Orringer DA. Supratentorial hemispheric ependymomas: an analysis of 109 adults for survival and prognostic factors. J Neurosurg. 2016 Jan 8:1-9. [Epub ahead of print] PubMed PMID: 26745489. )). ===2009=== From three adult patients with supratentorial ependymomas, two patients had tumors of WHO Grade III (anaplastic variant) and one had tumor of WHO Grade II. In all patients, gross total resection was achieved. Additional radiation therapy was administered in the Grade III patients. Twenty-four-month follow-up in case 1 yielded no tumor recurrence and no requirement of adjuvant chemotherapy. In case 2, tumor recurred with leptomeningeal gliomatosis by 6 months. Addition of platinum-based chemotherapy did not improve long-term survival; the patient succumbed to the disease after 14 months. In case 3 (WHO Grade II), no radiation therapy was required. Tumor did not recur during the 42-month follow-up. Gross total resection was achieved in all patients with hemispheric supratentorial WHO Grade II or Grade III ependymomas with additional radiation therapy for Grade III variants. All patients require initial close serial imaging and follow-up. The role of chemotherapy is still uncertain but may be necessary in younger patients who may have tumors that behave more like the pediatric ependymomas ((Niazi TN, Jensen EM, Jensen RL. WHO Grade II and III supratentorial hemispheric ependymomas in adults: case series and review of treatment options. J Neurooncol. 2009 Feb;91(3):323-8. doi: 10.1007/s11060-008-9717-z. Epub 2008 Nov 1. Review. PubMed PMID: 18974933. )).