Ependimoma intracraneal: apuntes clínicos, moleculares y anatomopatológicos / Intracranial ependymoma: clinical, molecular and pathological notes

RESUMEN Los ependimomas surgen de las células ependimarias que revisten los ventrículos y los pasajes en el encéfalo y el centro de la médula espinal. Las células ependimarias producen líquido cefalorraquídeo. Se decidió la realización de una revisión acerca del ependimoma intracraneal teniendo en cuenta que no existe artículo nacional que trate este tema, siendo la mayoría de los trabajos consultados referentes a la misma variante histológica pero en localización espinal, cuyo objetivo es describir la características clínicas, moleculares y anatomopatológicas del ependimoma intracraneal. Se realizó la búsqueda de artículos en revistas de las bases de datos: PubMed, Scielo y EBSCO. La búsqueda se limitó a artículos con el texto completo, publicados fundamentalmente en los últimos cinco años. El ependimoma intracraneal es un tumor frecuente en la edad pediátrica, sus manifestaciones clínicas dependen de su localización, presenta una gran diversidad molecular y anatomoptológica (AU).

SUMMARY Ependymomas arise from ependymal cells that line the ventricles and passages in the brain and center of the spinal cord. Ependymal cells produce cerebrospinal fluid. It was decided to conduct a review about intracranial ependymoma taking into account that there is no national article dealing with this issue, with most of the works consulted referring to the same histological variant but in spinal location, whose objective is to describe the clinical characteristics, Molecular and pathological pathways of intracranial ependymoma. We searched articles in journals of the databases: PubMed, Scielo and EBSCO. The search was limited to articles with the full text, published mainly in the last five years. Intracranial ependymoma is a frequent tumor in the pediatric age, its clinical manifestations depend on its location, it has a great molecular and anatomoptological diversity (AU).

Ependimoma mixopapilar dorsolumbar: reporte de un caso / Ependymoma myxopapillary dorsolumba: case report

Se presenta un caso con diagnóstico de Ependimoma mixopapilar de localización dorso lumbar con cuadro de presentación de paraparesia progresiva y que recibió tratamiento quirúrgico y oncológíco. La evolución no fue satisfactoria a pesar de mejorar el estado neurológico motor, por su grado de extensión y adherencia a estructuras nerviosas y óseas.

Síndrome lumbociático de etiología distinta a hernia discal / Etiology of lumbosciatic syndrome different from lumbar herniated disk

La causa más frecuente de lumbociática es la hernia de disco y la patología asociada de estenosis foraminal, espondilolistesis y entesopatía de la articulación facetaria, incluyendo quistes sinoviales. Existen una serie de condiciones que pueden presentar un cuadro clínico similar, y el problema es detectar estas causas infrecuentes en un universo muy grande de pacientes con patología discal. Esto crea una situación potencialmente peligrosa, en la cual se podría interpretar la sintomatología secundaria, por ejemplo a un tumor, como producida por una hernia discal, por otro lado asintomática. En base a una historia clínica cuidadosa y al uso racional de los exámenes complementarios, se puede sospechar aquellos casos que pudieran albergar esta patología de baja incidencia, pero de gran importancia clínica. Se revisan las causas más importantes que pueden provocar un síndrome lumbociático y que deben incluirse en el diagnóstico diferencial, como el síndrome piriforme, tumores intradurales y del nervio ciático y fístulas durales.

The most frequent etiology of sciatic pain is herniation of the nucleus pulposus and associated entesopathic diseases, including synovial cysts. There are several conditions that can present with a similar clinical picture, and the clinician is confronted with the problem of detecting this infrequent occurrences. This creates a potentially dangerous condition of thinking that an asymptomatic disc herniation is causing the symptoms that are originated higher by a tumor for example. With a careful history and judicious use of ancillary examinations, specially NMR, most of the cases can be suspected. The principal causes of non-discal sciatica are reviewed, including piriform syndrome, tumors of the spine and sciatic nerve, and dural fistulae.

Vertebrobasilar artery dissection complicating cervico-dorsal intramedullary Ependymoma: a case report

Vertebral artery dissection [VAD] is an important cause of posterior circulation stroke in young adults. It could be either spontaneous or traumatic; initial symptoms are often non-specific, and diagnostic arteriography is not performed until neurological deficits are obvious. We describe a rare case of a patient who presented with cervico-dorsal intramedullary ependymoma, and developed brain stem infarction caused by vertebrobasilar artery dissection during her postoperative period, which was diagnosed by Magnetic Resonance Imaging [MRI] and Magnetic Resonance Angiography [MRA]. Although vertebrobasilar artery dissection is rare, it should be considered in the differential diagnosis of brain stem infarction, and urgent MRI / MRA should be done as it is a reliable diagnostic tool and less invasive compared to cerebral angiogram

Recurrent intramedullary cervical ependymal cyst.

We report a rare case of high cervical intramedullary ependymal cyst in a young boy. This was associated with atlantoaxial dislocation. After partial removal and marsupialization, the cyst recurred and needed radical total resection.

Extradural myxopapillary ependymoma with sacral osteolysis--a case report.

Extradural ependymomas arising from filum terminale externa in the sacrococcygeal region are very rare. Since 1937, out of 58 cases reported in English literature, only 13 cases of tumor located presacrally in the retrorectal space, have been reported. The authors report a 38 yrs old male with a primary ependymoma of the sacrococcygeal region causing extensive sacral destruction.

Esclerose múltipla, ependimoma medular e meningioma intracraniano: relato de caso / Multiple sclerosis, spinal cord ependymoma and intracranial meningioma: case report

Relatamos a associaçäo impar entre esclerose múltipla (EM), tumor medular e tumor intracraniano em uma paciente de 63 anos de idade e com EM há dez anos com evoluçäo em surtos de remissäo e exacerbaçäo. Havia melhora dos sintomas com o uso de corticosteráides. Em 1997 apresentou paraparesia crural e do membro superior direito, de instalaçäo progressiva e que näo respondeu à corticoterapia. A ressonância magnética da coluna cervical evidenciou tumor intramedular, que se revelou um ependimoma, e a do crânio, a presença de meningioma parietal à esquerda. Ressaltamos a associaçäo incomum entre tumores do sistema nervoso central e EM e enfatizamos a necessidade de investigaçäo clínica e por imagem diante de uma manifestaçäo ou evoluçäo clínica incomum no curso da doença

Association of lower cranial nerve schwannoma with spinal ependymoma in ? NF2.

A 15 year old male, who had earlier been operated for intraspinal intramedullary ependymoma, subsequently developed a right cerebello pontine (CP) angle mass. A diagnosis of right CP angle ependymoma was considered, in view of established histology of previously operated spinal lesion. Histopathological examination of the well defined extra-axial mass, which was attached with ninth cranial nerve, however revealed a schwannoma. A diagnosis of Neurofibromatosis-2 (NF2) is strongly suspected, because of well established fact, that the spinal ependymomas may have association with lower cranial nerve schwannomas in NF2. Cranial and spinal MRI screening for early diagnosis of associated, asymptomatic lesions, in suspected cases of NF2, particularly in children, is recommended.

Audiometria de tronco cerebral (BERA) e eletrococleografia (ECoG) em uma criança operada de ependimoma intracraniano / Brain stem audiometry (BERA) and electrocochleography (ECoG) in a operated child of intracranial ependynoma

Os autores apresentam um caso de ependinoma intracraniano, localizado na regiäo do IV ventrículo, em criança de 3 anos. Após a remoçäo cirúrgica do tumor, houve perda auditiva unilateral, comprovada pela audiometria tonal limiar. A pesquisa da possível localizaçäo da lesäo do sistema auditivo através da eletrococleografia e audiometria do tronco cerebral mostrou que a lesäo se localizava no tronco cerebral. Este caso ilustra bem a importância da ECoG e do BERA no topo diagnóstico de lesöes de aparelho auditivo.

Tuberous sclerosis presenting as an intraventricular tumour.

We report a case of tuberous sclerosis in an 8 year old girl presenting for the first time with features of raised intracranial pressure due to a large intraventricular tumour. Occurrence of these tumours in children with tuberous sclerosis justifies cranial computerised tomography as a screening procedure to detect these tumours early.