Purely Cortical Anaplastic Ependymoma

Abstract

Ependymomas are glial tumors derived from ependymal cells lining the ventricles and the central canal of the spinal cord. It may occur outside the ventricular structures, representing the extraventicular form, or without any relationship of ventricular system, called ectopic ependymona. Less than fifteen cases of ectopic ependymomas were reported and less than five were anaplastic. We report a rare case of pure cortical ectopic anaplastic ependymoma.

Supratentorial Extraventricular Anaplastic Ependymoma Presented With Headache in Pregnancy: Case Report and Review of the Literature

“…We found 20 reported cases or case series that had the same characteristics as this study prior to June 2014 (Table 1) [6,8,9,12,[14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29]. Only one reported case dealt with pregnancy [4,12,13]. We report a case of a patient with an SEAE that presented with a pregnant and purely cystic appearance on MRI and computed tomography (CT).…”

“…Supratentorial ependymomas in adults are unusual lesions, often found to be of the anaplastic variant [16]. While the tumors that form in the lateral ventricle expand toward the cerebral tissue, tumors may also sometimes appear purely in a cortical location [4,12,17]. The described tumor (SEAE) is extraordinarily rare.…”

“…In the literature, the 5-year survival rate without recurrence is 75%, but, in those characterized as aggressive, this period is less than 31% [21]. The severe negative prognostic criteria are determined as: a young age, incomplete tumor resection, histological anaplasia, and supratentorial localization [12,17]. It is reported that it is necessary to carry out an as-large-as-possible resection for SEAE characterized as anaplastic to reduce or eliminate recurrence [12].…”

“…The severe negative prognostic criteria are determined as: a young age, incomplete tumor resection, histological anaplasia, and supratentorial localization [12,17]. It is reported that it is necessary to carry out an as-large-as-possible resection for SEAE characterized as anaplastic to reduce or eliminate recurrence [12]. But it relapsed after all resections.…”

“…According to the World Health Organization's (WHO) 2007 classification of central nervous system tumors, low-grade ependymoma was grade 2 and anaplastic ependymoma was grade 3 [9,10]. Ependymomas located outside the ventricles are called ectopic, cortical, lobar or extraventricular ependymomas [11,12].…”