An ependymoma is anaplastic if the cells grow very quickly and are significantly unusual in shape. Adult intracranial ependymoma is a relatively rare brain tumour entity, accounting for 25% of all intracranial neoplasms. 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. Ependymoma introduction any tumor that arises from the glial cells in the brain is called a glioma. Combined tanycytic ependymoma and subependymoma, who grade ii.
Know the causes, symptoms, treatment, prognosis of ependymoma. 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. Ependymomas are soft, red, or grayish in color that may possibly comprise of mineral or cysts calcifications. 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. This variant is preferentially located in the supratentorial compartment of the brain. Tanycytic ependymoma is the rarest variant of ependymoma and occurs primarily in the spinal cord. 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. Tanycytic ependymoma has a predilection for the spinal cord. Less than 30 have been reported to date, most as small case reports. Cerebellar ependymoma with overlapping features of clear.
Neurological imaging suggested hemangioblastoma hb. Tanycytic cells are common progenitor cells of both ependymal cells and astrocytes. The pertinent prognostic factors as well as the pattern of recurrence remain to be elucidated. Review article ependymoma diagnosis and treatment progress. The ependyma is the epithelial lining of the brain ventricles and the central canal of. Ependymoma definition of ependymoma by medical dictionary. 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. 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.
Childhood ependymoma treatment pdqhealth professional. Ependymoma is a rare tumor that arises from ependymal cells lining the ventricles and passageways in the spinal cord and brain. 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. Supratentorial tanycytic ependymoma in an adult male. Lowrisk complete resection, m0 and age tanycyticvariant of ependymomas.
Spinal cord ependymomas, which are often the myxopapillary variant, tend to cause. Subependymomas are uncommon lesions that share the benign features of myxopapillary ependymomas. Tanycytic ependymomas usually carry better prognosis as compared to other variant of ependymomas and astrocytomas. Clear cell ependymomadisplays an oligodendrogliallike appearance with perinuclear halos. Ependymoma brain and spinal cord tumours cancer research uk. Senile tanycytic ependymoma that arises from the spinal cord is extremely rare. This tumor is composed of clusters of elongated cells forming nuclear dense zones and streaming cell processes forming fibrillary zones. Common symptoms include headaches and feeling or being sick. Ependymoma brain tumors arise from a special type of cell in the central nervous system cns known as the ependymal cells. Only 9 ventricular and 5 subcortical tanycytic ependymoma have been reported in the literature. 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. Tanycytic ependymoma who grade ii is a rare subtype of ependymoma arising from tanycytes. According to several recent reports, the prognostic value of karnofski performance status, tumour location, the extent of. Tanycytic ependymoma is a rare form of ependymoma that usually arises in the intramedullary spine.
Ependymoma can affect children, which is intracranial in about 90% of cases. Glial cells provide support and protection for the nerve cells, or neurons, in the brain. Webpathology is a free educational resource with 10297 high quality pathology images of benign and malignant neoplasms and related entities. Furthermore, there are even rarer variants of ependymomas that can include cellular, papillary, clear cell, and tanycytic subtypes. We present an unusual supratentorial subcortical tanycytic ependymoma in a 17year old male presenting with seizures. An ependymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Their occurrence seems to peak at age 5 years and then again at age 35.
Computed tomography and magnetic resonance imaging revealed a mass in the cerebellum. Jan 11, 2016 tanycytic ependymoma is an unusual morphological variant of who grade ii ependymoma, typically arising from the cervical or thoracic spinal cord. Anaplastic ependymoma hitskk87 and hitskk92 19871992, 19921997 hitskk87. 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. Radiation oncologycnsependymoma wikibooks, open books. Ependymomas were first discovered by bailey in the year 1924. 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. Ependymomas arise from ependymal cells that line the ventricles of the brain and the center of the spinal cord. You will learn about how doctors describe the growth or spread of ependymoma. The positive samples were usually hypercellular, with cohesive epithelioid cells.
Cerebellar ependymoma with overlapping features of clearcell. Tanycytic ependymoma is an unusual morphological variant of who grade ii ependymoma, typically arising from the cervical or thoracic spinal cord. Usually, in pediatric cases the location is intracranial, while in adults it is spinal. In addition to the classic and anaplastic types, myxopapillary and tanycytic variants of ependymoma have also been described in the spinal cord. Case report senile tanycytic ependymoma of the spinal cord. Ependymoma brain tumors can arise in the brain intracranial or the spinal cord. Ependymoma grade ii is the most common of the ependymal tumors and typically arises within or near the ventricular system. An ependymoma is a rare type of primary brain or spinal cord tumors. This uncommon ependymoma variant is composed of elongated, spindleshaped cells with thin eosinophilic fibrillary processes see the following image. Lowrisk complete resection, m0 and age ependymoma, one of the first symptoms you may notice is that he has a larger than usual head. What is ependymomatypescausessymptomstreatmentprognosis. True ependymal rosettes are absent, and perivascular rosettes are inconspicuous. Ependymoma is a rare type of brain tumour called a glioma.
If your baby has an ependymoma, one of the first symptoms you may notice is that he has a. They start from ependymal cells in the brain or spinal cord. The common location of intracranial ependymomas is the fourth ventricle. Tanycytic ependymomathe rarest form of grade ii ependymoma. Tanycytic ependymoma is a rare fibrillary variant of ependymoma with a predilection for the spinal cord. 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. 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. We present an unusual supratentorial subcortical tanycytic ependymoma in a 17year old male. The authors report a 50yearold man with a cervical tanycytic ependymoma that was initially thought to be a.
In these lesions, the classic ependymal rosettes and perivascular pseudorosettes are replaced by more fibrillar cells. Ependymomas are found throughout the central nervous system in the supratentorial top of the head, posterior fossa back of the head and spinal compartments. Cerebrospinal fluid cytology in patients with ependymoma. 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. 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.
Their name reflects the morphological similarlity of these tumor cells to the tanycytes. Tanycytic ependymoma is a rare variant of ependymoma and treatment guidelines are not established. Tanycytic ependymomas are histological variants of ependymomas, usually found in the spinal cord 1. It has a unique histology emphasized by the inconspicuous ependymal pattern of cells and close resemblance to schwannoma and astrocytoma. Tanycytic ependymoma is an uncommon fibrillar variant of ependymoma characterized by streams of piloid, or hairlike, cells having ependymal nuclei. We present an unusual supratentorial subcortical tanycytic ependymoma in a. Disease bioinformatics research of ependymoma has been linked to neoplasms, brain neoplasms, astrocytoma, glioma, medulloblastoma. The brain controls vital functions such as memory and learning, emotion, and the senses hearing, sight, smell, taste, and touch.
Ependymoma cells are known to occasionally exfoliate into. Jan 05, 2018 tanycytic ependymoma has a predilection for the spinal cord. Childhood ependymoma is a disease in which malignant cancer cells form in the tissues of the brain and spinal cord. Ependymoma accounts for 4% adult central nervous system tumors, and 10% central nervous system tumors in children 2. Diagnostic imaging demonstrated a right subcortical pa rietooccipital heterogeous expansive lesion, with growth. Of the 8 patients with positive andor suspicious diagnoses, 5 ependymomas exhibited anaplastic features and 1 tumor was a myxopapillary ependymoma. Ependymoma prognosis, survival rate, symptoms, anaplasti. In childhood ependymoma, cancer forms in the tissues of the brain and spinal cord. In summary, ependymal tumors of the sella are rare, poorly understood, and generally benign tumors. The spinal cord is made up of bundles of nerve fibers that connect the brain with nerves in most parts of the body. Ependymoma is a comparatively rare type of tumor of the central nervous system cns including, the brain and the spinal cord. 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.
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. Diagnosis as a world health organization who grade ii tanycytic ependymoma was supported by its appearance on evaluation of the permanent. A unique variant of ependymoma, myxopapillary ependymoma, occurs in the distal spinal cord filum terminale and has. Our patient had a cpa mass that was a variant of ependymoma known as tanycytic ependymoma that mimicked vestibular schwannoma. The ependyma is the epithelial lining of the brain ventricles and the central canal of 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 genetic and rare diseases information center. Radiation oncologycnsependymoma wikibooks, open books for. Only two other cases of subcortical tanycytic ependymoma have been reported. Symptoms of ependymomas depend on whether the tumour is in the brain or the spinal cord. They do not arise from tanycytes which are located primarily in the floor of the third ventricle. Imaging and histology of clearcell ependymoma and cerebellumbased hemangioblastoma are similar. Based on the histological characteristics of the tumor cells, ependymomas can be classified into further subtypes. Ependymomas make up about 5% of adult intracranial gliomas and up to 10% of childhood tumors of the central nervous system cns. The study of ependymoma has been mentioned in research publications which can be found using our bioinformatics tool below. They are thought to develop from certain cells that normally mature. The authors report a 50yearold man with a cervical tanycytic ependymoma that was initially thought to be a schwannoma. A 62yearold chinese woman presented with an intermittent headache of 8 years duration. Amongst the 9 ventricular cases, only one tumor arose from the third ventricle. Papillary ependymomaforms linear, epitheliallike surfaces along cerebrospinal fluid exposures.
Although the literature deals extensively with pathological features of this tumour entity, imaging features have not been well characterised. Tanycytic ependymoma is a rare subtype of ependymoma that most commonly occurs in the brain. Radiotherapy for a supratentorial tanycytic ependymoma. Rela fusionpositive a new entity in 2016 update who grade iii. 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. 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.
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