They start from ependymal cells in the brain or spinal cord. The present study reported the case of a 65yearold woman who presented with a 4day history of numbness in both lower limbs accompanied by an in. Ependymomas arise from ependymal cells that line the ventricles of the brain and the center of the spinal cord. Combined tanycytic ependymoma and subependymoma, who grade ii. Ependymoma prognosis, survival rate, symptoms, anaplasti. Childhood ependymoma is a disease in which malignant cancer cells form in the tissues of the brain and spinal cord. Diagnosis as a world health organization who grade ii tanycytic ependymoma was supported by its appearance on evaluation of the permanent. Of the 8 patients with positive andor suspicious diagnoses, 5 ependymomas exhibited anaplastic features and 1 tumor was a myxopapillary ependymoma. Tanycytic ependymoma who grade ii is a rare subtype of ependymoma arising from tanycytes. Anaplastic ependymoma hitskk87 and hitskk92 19871992, 19921997 hitskk87. Tanycytic ependymoma is a rare fibrillary variant of ependymoma with a predilection for the spinal cord. True ependymal rosettes are absent, and perivascular rosettes are inconspicuous. The common location of intracranial ependymomas is the fourth ventricle.
The objective of our case report is to document the radiological and typical ependymal features of the neoplasm and to increase awareness in oncology community. Based on the histological characteristics of the tumor cells, ependymomas can be classified into further subtypes. Ependymoma brain tumors can arise in the brain intracranial or the spinal cord. Ependymoma brain and spinal cord tumours cancer research uk. In addition to the classic and anaplastic types, myxopapillary and tanycytic variants of ependymoma have also been described in the spinal cord. A unique variant of ependymoma, myxopapillary ependymoma, occurs in the distal spinal cord filum terminale and has. Glial cells provide support and protection for the nerve cells, or neurons, in the brain. Ependymoma brain tumors arise from a special type of cell in the central nervous system cns known as the ependymal cells. Their occurrence seems to peak at age 5 years and then again at age 35. Usually, in pediatric cases the location is intracranial, while in adults it is spinal. We present an unusual supratentorial subcortical tanycytic ependymoma in a 17year old male presenting with seizures. Feb 14, 20 tanycytic ependymoma is a world health organization who grade ii tumor with histological characteristics distinct from the typical features of commonly encountered ependymomas. Cerebrospinal fluid cytology in patients with ependymoma.
Jan 11, 2016 tanycytic ependymoma is an unusual morphological variant of who grade ii ependymoma, typically arising from the cervical or thoracic spinal cord. Rela fusionpositive a new entity in 2016 update who grade iii. Common symptoms include headaches and feeling or being sick. Case report senile tanycytic ependymoma of the spinal cord. It has a unique histology emphasized by the inconspicuous ependymal pattern of cells and close resemblance to schwannoma and astrocytoma. Cerebellar ependymoma with overlapping features of clear. Ependymoma is a rare tumor that arises from ependymal cells lining the ventricles and passageways in the spinal cord and brain. An ependymoma is a rare type of primary brain or spinal cord tumors. Ependymoma grade ii is the most common of the ependymal tumors and typically arises within or near the ventricular system. Webpathology is a free educational resource with 10297 high quality pathology images of benign and malignant neoplasms and related entities.
Jan 05, 2018 tanycytic ependymoma has a predilection for the spinal cord. Although the literature deals extensively with pathological features of this tumour entity, imaging features have not been well characterised. Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting other parts of the body. Tanycytic ependymoma is an uncommon fibrillar variant of ependymoma characterized by streams of piloid, or hairlike, cells having ependymal nuclei. Our patient had a cpa mass that was a variant of ependymoma known as tanycytic ependymoma that mimicked vestibular schwannoma. Radiotherapy for a supratentorial tanycytic ependymoma. Subependymomas are uncommon lesions that share the benign features of myxopapillary ependymomas. We present an unusual supratentorial subcortical tanycytic ependymoma in a 17year old male. Ependymoma introduction any tumor that arises from the glial cells in the brain is called a glioma. Tanycytic ependymomas usually carry better prognosis as compared to other variant of ependymomas and astrocytomas.
Misinterpretation as schwannoma or astrocytoma is a diagnostic problem and welldocumented cases are scarce. This being the case, ependymoma of the neurohypophysis may be pituicytederived and a variant of pituicytoma. Ependymoma arise from the ependymal cells of the cerebral ventricles, corticle rests and central canal of the spinal cord, and manifest with variable symptoms such headache, vomiting, seizures, focal neurological signs and loss of vision and can cause obstructive hydrocephalus in some cases. Diagnostic imaging demonstrated a right subcortical pa rietooccipital heterogeous expansive lesion, with growth. They develop from cells that line both the hollow cavities of the brain and the canal containing the spinal cord, but they usually arise from the floor of the. The positive samples were usually hypercellular, with cohesive epithelioid cells. Tanycytic ependymoma is an unusual morphological variant of who grade ii ependymoma, typically arising from the cervical or thoracic spinal cord. The brain controls vital functions such as memory and learning, emotion, and the senses hearing, sight, smell, taste, and touch. Disease bioinformatics research of ependymoma has been linked to neoplasms, brain neoplasms, astrocytoma, glioma, medulloblastoma. Tanycytic ependymomas are histological variants of ependymomas, usually found in the spinal cord 1. This variant is preferentially located in the supratentorial compartment of the brain. Supratentorial tanycytic ependymoma in an adult male.
The occurrence of ependymoma in adults usually peaks between the ages of 35 to 45, with onethird occurring in the cranium and twothirds in spinal cord 3, while in pediatric cases, ependymoma occurs usually under the age of. Ependymoma genetic and rare diseases information center. Ependymomas were first discovered by bailey in the year 1924. They do not arise from tanycytes which are located primarily in the floor of the third ventricle. The study of ependymoma has been mentioned in research publications which can be found using our bioinformatics tool below. Lowrisk complete resection, m0 and age ependymoma, one of the first symptoms you may notice is that he has a larger than usual head. Because of this derivation, tanycytic ependymomas were presumed to typically be found in the spinal cord where the raphe are abundant in ependymoglia and tanycytes. Less than 30 have been reported to date, most as small case reports. Clear cell ependymomadisplays an oligodendrogliallike appearance with perinuclear halos. The tanycyticvariant of ependymomas was first characterized in detail by friede and pollak as having an appearance more similar to a common ancestor of both ependymal cells and astrocytes known as ependymoglia or tanycytes. The ependyma is the epithelial lining of the brain ventricles and the central canal of. A 62yearold chinese woman presented with an intermittent headache of 8 years duration. Only two other cases of subcortical tanycytic ependymoma have been reported. They are thought to develop from certain cells that normally mature.
Radiation oncologycnsependymoma wikibooks, open books for. In these lesions, the classic ependymal rosettes and perivascular pseudorosettes are replaced by more fibrillar cells. Senile tanycytic ependymoma that arises from the spinal cord is extremely rare. Tanycytic cells are common progenitor cells of both ependymal cells and astrocytes. Ependymoma cells are known to occasionally exfoliate into. The spinal cord is made up of bundles of nerve fibers that connect the brain with nerves in most parts of the body. Tanycytic ependymomas are a very rare or at least very rarely diagnosed fibrillary variant of ependymomas that arise from primitive tanycytes and are distinguishable based on ultrastructural and. You will learn about how doctors describe the growth or spread of ependymoma. Ependymoma accounts for 4% adult central nervous system tumors, and 10% central nervous system tumors in children 2. The pertinent prognostic factors as well as the pattern of recurrence remain to be elucidated. Adult intracranial ependymoma is a relatively rare brain tumour entity, accounting for 25% of all intracranial neoplasms.
Furthermore, there are even rarer variants of ependymomas that can include cellular, papillary, clear cell, and tanycytic subtypes. If your baby has an ependymoma, one of the first symptoms you may notice is that he has a. Tanycytic ependymoma has a predilection for the spinal cord. This tumor is composed of clusters of elongated cells forming nuclear dense zones and streaming cell processes forming fibrillary zones. According to several recent reports, the prognostic value of karnofski performance status, tumour location, the extent of. Tanycytic ependymoma is a rare subtype of ependymoma that most commonly occurs in the brain. Review article ependymoma diagnosis and treatment progress. Tanycytic ependymoma is a rare form of ependymoma that usually arises in the intramedullary spine. The authors report a 50yearold man with a cervical tanycytic ependymoma that was initially thought to be a schwannoma. Only 9 ventricular and 5 subcortical tanycytic ependymoma have been reported in the literature.
It can occur in both adults and children at any age, but in adults the frequency of this disease is more visible than in children. Cerebellar ependymoma with overlapping features of clearcell. Ependymoma definition of ependymoma by medical dictionary. Tanycytic ependymoma is a rare variant of ependymoma and treatment guidelines are not established. Ependymomas make up about 5% of adult intracranial gliomas and up to 10% of childhood tumors of the central nervous system cns. Mar 01, 2014 ependymoma arise from the ependymal cells of the cerebral ventricles, corticle rests and central canal of the spinal cord, and manifest with variable symptoms such headache, vomiting, seizures, focal neurological signs and loss of vision and can cause obstructive hydrocephalus in some cases. Symptoms of ependymomas depend on whether the tumour is in the brain or the spinal cord. Fibrillar variant of ependymoma discrete margin with surrounding tissue features of ependymoma and astrocytoma elongated spindle cells with round oval nuclei with distinctly light and dark regions of chromatin similar to ependymoma and marked fibrillarity similar to astrocytoma. Ependymoma is a comparatively rare type of tumor of the central nervous system cns including, the brain and the spinal cord. Ependymomas are soft, red, or grayish in color that may possibly comprise of mineral or cysts calcifications. Fibrillar variant of ependymoma discrete margin with surrounding tissue features of ependymoma and astrocytoma elongated spindle cells with round oval nuclei with distinctly light and dark regions of chromatin similar to ependymoma and marked fibrillarity similar to.
Our patient had a cpa mass that was a variant of ependymoma known as tanycytic ependymoma that mimicked vestibular schwannoma radiologically and during intraoperative pathologic examination. Computed tomography and magnetic resonance imaging revealed a mass in the cerebellum. Tanycytic ependymoma is the rarest variant of ependymoma and occurs primarily in the spinal cord. Anaplastic ependymoma is a type of ependymoma, which is a tumor that forms when cells in the central nervous system including the brain and spinal cord begin to multiply rapidly. Ependymoma can affect children, which is intracranial in about 90% of cases. Lowrisk complete resection, m0 and age tanycyticvariant of ependymomas. Ependymoma is a rare type of brain tumour called a glioma.
Papillary ependymomaforms linear, epitheliallike surfaces along cerebrospinal fluid exposures. The authors report a 50yearold man with a cervical tanycytic ependymoma that was initially thought to be a. An ependymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Tanycytic ependymomathe rarest form of grade ii ependymoma. Childhood ependymoma treatment pdqhealth professional. The ependyma is the epithelial lining of the brain ventricles and the central canal of the spinal cord. Their name reflects the morphological similarlity of these tumor cells to the tanycytes. Ependymomas develop in all age groups but occur more frequently in children, being the most common spinal cord tumor in children and adolescents ages 019 years with an incidence of 23. What is ependymomatypescausessymptomstreatmentprognosis. Feb 02, 2018 myxopapillary ependymomas are considered a biologically and morphologically distinct variant of ependymoma, occurring almost exclusively in the region of the cauda equina and behaving in a more benign fashion than grade ii ependymoma. Amongst the 9 ventricular cases, only one tumor arose from the third ventricle.
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