Ependymoma with Intraorbital Extracerebral Recurrence: Case Report

Ependymomas are rare neuroepithelial tumors that originate from a type of glial cell called ependymal cell. In general, they correspond to 1.2 to 7.8% of all intracranial neoplasms, and to2 to 6%of all gliomas. Although it corresponds only to2 to 3%of all primary brain tumors, ependymoma is the fourthmost common cerebral neoplasmin children, especially in children younger than 3 years of age.1,2 In patients younger than 20 years of age, the majority (90%) of ependymomas are infratentorial,more precisely from the IV ventricle. In spite of this, in adults, medullary ependymomas are more frequent (60%). In this context, supratentorial and extraventricular ependymomas, as in the case reported in the present article, are infrequent in both adults and children.1,2 Both sexes are equally affected.3 Recurrence of intracranial ependymomas occurs in almost 50% of the cases, and the followup outcome is not favorable.4 In another perspective, the recurrence of extracerebral ependymomas is extremely rare, and even more unusual in the intraorbital site, as it occurred in the case in question.

Supratentorial Ependymoma:Experience Of A Neurosurgeon

Background-Ependymoma account for 5 to 10 % of all brain tumors in young population however majority occur in infratentorial location and Supratentorial Ependymoma (STE) are extremely rare . Aim of this original article is to describe demographic , clinical features and surgical outcome of these rare tumors operated by Neurosurgeon DSM .Materials and Method-10 cases of STE were operated by a single Neurosurgeon DSM from May 2017 to January 2019 at a tertiary care hospital.Results-Gross total resection was performed in 8 cases and Near total resection was performed in 2 cases . Recurrence was seen in 3 cases during study period and 2 cases required resurgery Histopathology was anaplastic Ependymoma WHO grade 3 in all the cases.Conclusion-STE have a better prognosis as compared to infratentorial Ependymoma in view of good chance of gross total excision .

A Case of Recurrent Supratentorial Extraventricular Anaplastic Ependymoma in Adult

Supratentorial extraventricular anaplastic ependymoma (SEAE) in adults is a relatively rare intracranial tumor. Because of the very low prevalence, only a few cases have been reported. According to a recent study, SEAE is associated with a poor prognosis and there is no definite consensus on optimal treatment. We report a case of an adult SEAE patient who had no recurrence until seven years after a gross total resection (GTR) followed by conventional radiotherapy. A 42-year-old male had a persistent mild headache, left facial palsy, dysarthria, and left hemiparesis. Preoperative neuroimaging revealed an anaplastic astrocytoma or supratentorial ependymoma in the right frontal lobe. A GTR was performed, followed by adjuvant radiotherapy. Histologic and immunohistochemical results revealed anaplastic ependymoma. After seven years of initial therapy, a regular follow-up MRI showed a 3-cm-sized partially cystic mass in the same area as the initial tumor. The patient underwent a craniotomy, and a GTR was performed. Histopathologic examination revealed recurrence of the SEAE. External radiotherapy was performed. The patient has been stable without any disease progression or complications for 12 months since the surgery for recurrent SEAE.

Ependimoma anaplásico supratentorial cortical. Reporte de caso / Cortical supratentorial anaplastic ependymoma. Case report

Introducción: Los ependimomas son tumores neuroepiteliales que representan del 2 al 9% de todos los tumores del SNC. Existe una variante ectópica que se ubica alejada de las células ependimarias que darían su origen. Esta última variante se presenta más frecuentemente en el compartimiento supratentorial. Objetivo: Reporte de un caso de ependimoma anaplásico occipital cortical y revisión de la literatura de esta entidad con una baja casuística mundial.Reporte de caso: Masculino de 35 años, ex tabaquista, cefalea de 2 meses de evolución y disminución de la agudeza visual. Al examen se constató cefalea holocraneana, mareos y hemianopsia homónima derecha por confrontación. Fondo de ojo (FO): edema de papila bilateral, con hemorragias peripapilares en ojo izquierdo. Estudios neuroradiológicos: lesión cortical occipital izquierda con refuerzo homogéneo post contraste, limites netos y abundante edema perilesional. Screening oncológico negativo. Resección quirúrgica, la pieza quirúrgica presentaba buen plano de clivaje, coloración blanquecina y consistencia duroelástica. Hallazgos vinculables con Ependimoma Anaplásico. Se le realizó tratamiento adyuvante STUPP. Screening de siembra metastásica craneoespinal negativo. Al año post tratamiento no se evidenció recidiva de la lesión, con mejoría del CVC y sin edema de papila en el FO control. Conclusiones: Los ependimomas anaplásicos supratentoriales corticales son una entidad rara. En pacientes jóvenes con imágenes que evidencian lesión cortical única con realce homogéneo post contraste debe considerarse a esta entidad dentro de los diagnósticos diferenciales. La resección quirúrgica es el tratamiento de elección, seguido de RT, en variantes malignas o con resección incompleta.

Introduction: Ependymomas are neuroepithelial tumors, representing 2 to 9% of all CNS tumors. There is an ectopicvariant located away from the ependymal cells that wouldoriginate. This last variable occurs more frequently in thesupratentorial compartment.Objectives: Case report of an occipital cortical anaplasticependimoma. Literature review of this entity with a low globalcasuistry. Case report: Male 35 years, former smoker, headache 2 monthsof evolution and decreased visual acuity. Physical examination found holocraneal headache, dizziness and right homonymoushemianopia by confrontation. Ocular fundus (OF): bilateralpapilledema, peripapilar bleeding in left eye. Neuroradiological studies: left occipital cortical lesion with homogeneousreinforcement after contrast, net limits and abundant perilesionaledema. Negative oncological screening. Surgical resection, Thesurgical specimen showed good cleavage plane, whitish and firm,elastic consistency. Linkable findings with anaplasticependimoma. STUPP Adyuvant therapy was performed. Negativescreening of craniospinal metastatic seeding. During posttreatment year showed no recurrence of injury, with CVCimprovement, without papilledema in the ocular fundus control.Conclusions: Cortical supratentorial anaplastic ependimoma arerare. In young patients with images that show unique corticallesion with homogeneous reinforcement after contrast this entitymust be considered between differential diagnoses. Surgicalresection is the treatment of choice, followed by radiotherapy, inmalignant variants of incomplete resection.

Supratentorial Extraventricular Anaplastic Ependymoma Presenting with Repeated Intratumoral Hemorrhage

Supratentorial extraventricular anaplastic ependymomas are extremely rare. We report the case of a 23-year-old male with a supratentorial extraventricular anaplastic ependymoma that presented with repeated intratumoral hemorrhage. The patient was diagnosed with an intracerebral hematoma in the left occipital lobe and underwent operation. The hematoma did not reveal tumor cells, but a new tumor grew in the same location 5 years later. Magnetic resonance imaging showed a tumor with intratumoral hemorrhage. The patient underwent the tumor resection. Intraoperative findings showed that the tumor had no continuity with the ventricle. Histopathological examinations confirmed an anaplastic ependymoma. The spinal evaluation was unremarkable, and radiotherapy was administered to the left occipital lobe. Four years later, the tumor recurred at the cervicomedullary junction and T8-T9 levels. This case demonstrates that anaplastic ependymomas should be included in the differential diagnoses of supratentorial extraventricular tumors presenting with repeated intratumoral hemorrhage.

Postoperative radiotherapy for ependymoma

PURPOSE: To evaluated the patterns of failure, survival rate, treatment-related toxicity and prognostic factors in postoperative radiotherapy of patients with ependymoma. MATERIALS AND METHODS: Thirty patients who underwent surgery and postoperative radiotherapy for ependymoma between the period of June 1994 and June 2008 were reviewed retrospectively. The age of patients ranged from 21 months to 66 years (median, 19 years). Seventeen patients had grade II ependymoma, and 13 had grade III anaplastic ependymoma according to the World Health Organization grading system. The postoperative irradiation was performed with 4 or 6 MV photon beam with median dose of 52.8 Gy (range, 45 to 63 Gy), and radiation field including 2 cm beyond the preoperative tumor volume. Median follow-up period was 51 months (range, 12 to 172 months). RESULTS: Fourteen out of 30 (46.7%) patients experienced recurrence, and 12 of those died. Among those 14 patients who experienced recurrence, 11 were in-field and 3 were out-of-field recurrence. The 5-year overall survival (OS) and progression-free survival (PFS) rates were 66.7% and 56.1%, respectively. On univariate analysis, tumor grade was a statistically significant prognostic factor for OS and PFS. There were two complications after surgery and postoperative radiotherapy, including short stature and facial palsy on the left side. CONCLUSION: We observed good survival rates, and histologic grade was a prognostic factor affecting the OS and PFS. Almost all recurrence occurred in primary tumor site, thus we suggest further evaluation on intensity-modulated radiotherapy or stereotatic radiosurgery for high-risk patients such as who have anaplastic ependymoma.

Postoperative radiotherapy for ependymoma

PURPOSE: To evaluated the patterns of failure, survival rate, treatment-related toxicity and prognostic factors in postoperative radiotherapy of patients with ependymoma. MATERIALS AND METHODS: Thirty patients who underwent surgery and postoperative radiotherapy for ependymoma between the period of June 1994 and June 2008 were reviewed retrospectively. The age of patients ranged from 21 months to 66 years (median, 19 years). Seventeen patients had grade II ependymoma, and 13 had grade III anaplastic ependymoma according to the World Health Organization grading system. The postoperative irradiation was performed with 4 or 6 MV photon beam with median dose of 52.8 Gy (range, 45 to 63 Gy), and radiation field including 2 cm beyond the preoperative tumor volume. Median follow-up period was 51 months (range, 12 to 172 months). RESULTS: Fourteen out of 30 (46.7%) patients experienced recurrence, and 12 of those died. Among those 14 patients who experienced recurrence, 11 were in-field and 3 were out-of-field recurrence. The 5-year overall survival (OS) and progression-free survival (PFS) rates were 66.7% and 56.1%, respectively. On univariate analysis, tumor grade was a statistically significant prognostic factor for OS and PFS. There were two complications after surgery and postoperative radiotherapy, including short stature and facial palsy on the left side. CONCLUSION: We observed good survival rates, and histologic grade was a prognostic factor affecting the OS and PFS. Almost all recurrence occurred in primary tumor site, thus we suggest further evaluation on intensity-modulated radiotherapy or stereotatic radiosurgery for high-risk patients such as who have anaplastic ependymoma.

Recurrent posterior fossa anaplastic ependymoma with prominent chondroid metaplasia: A case report and review of literature.

We report an unusual case of a recurrent fourth ventricular anaplastic ependymoma with prominent chondroid metaplasia in a 16-year-old male. On initial presentation, the patient had a WHO Grade II tumor. However, at recurrence 1 year later, the tumor progressed to WHO Grade III tumor with more cellularity, necrosis and brisk mitotic activity. Chondroid metaplasia was present in both the initial and recurrent tumors.