Tanycytic ependymoma arising from the right lateral. The common location of intracranial ependymomas is the fourth ventricle. Tanycytic ependymoma with abundant ploemorphic giant cells is a rare variety of intramedullary spinal cord tumor arising from the conusfilum terminale region. Tanycytic ependymomathe rarest form of grade ii ependymoma. Radiation oncologycnsependymoma wikibooks, open books for. Tanycytic ependymoma is a rare fibrillary variant of ependymoma with preferentially located along the length of spinal cord. Key histological features are perivascular pseudorosettes and ependymal rosettes.
According to the who classification, ependymal tumors are classified as subependymomas, myxopapillary ependymomas, classic ependymomas, anaplastic ependymomas, and relafusionpositive ependymomas relaepn. An ependymoma is anaplastic if the cells grow very quickly and are significantly unusual in shape. Tanycytic ependymoma of the filum terminale with pleomorphic. Ependymoma accounts for 4% adult central nervous system tumors, and 10% central nervous system tumors in children 2.
Ependymoma programme synopsis university of birmingham. Rela fusionpositive a new entity in 2016 update who grade iii. Four cases of tanycytic ependymoma arising from the lateral ventricle have been reported in literature. A page dedicated to my daughter to show her there are long term. Radiotherapy for a supratentorial tanycytic ependymoma. True ependymal rosettes are absent, and perivascular rosettes are inconspicuous. The authors report a 50yearold man with a cervical tanycytic ependymoma that was initially thought to be a schwannoma. The signs and symptoms of childhood ependymoma are. Professional books consumer books acs professional.
Although the most recent version of the blue book is the 4 th edition from 2007, an update has been released in 2016 3, which should be considered a replacement for the earlier version at this point, a decision to undertake. Childhood ependymoma treatment pdqhealth professional. About 230 people under the age of 19 in the united states will be diagnosed with ependymoma this year. For the 2012 update of this guideline, medline, embase, cochrane and pubmed were searched using the terms ependymoma mesh heading, lowgrade ependymoma, subependymoma, and myxopapillary ependymoma, limited to clinical trials, clinical trials phase iiv, controlled clinical trials, randomized controlled trials, published from september 2009.
Cerebral and spinal cord tanycytic ependymomas in a young. Subependymomas are uncommon lesions that share the benign features of myxopapillary ependymomas. Papillary ependymomaforms linear, epitheliallike surfaces along cerebrospinal fluid exposures. Here, we present a rare case of cerebellar ependymoma with overlapping features of clearcell and tanycytic variants in an adult female, which mimicked hemangioblastoma hb on histology, thus necessitating immunohistochemistry ihc testing for definitive diagnosis. Tanycytic variant, often occurs in the thoracic tract of the spinal cord, and it is constituted by tanycites, that are typical elongated and bipolar cells that give to the tumor fibrillary aspects. Misinterpretation as schwannoma or astrocytoma is a diagnostic problem and welldocumented cases are scarce. A 42yearold man has undergone complete resection of a supratentorial tanycytic ependymoma, with no evidence of residual disease. In these lesions, the classic ependymal rosettes and perivascular pseudorosettes are replaced by more fibrillar cells. Histological differential diagnosis includes spindle. 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. Tanycytic ependymoma is an uncommon but wellrecognized variant of ependymoma. A unique variant of ependymoma, myxopapillary ependymoma, occurs in the distal spinal cord filum terminale and has.
The tumor was adjacent to the left lateral ventricle. I subependymoma or myxopapillary ependymoma, grade ii classic ependymoma with cellular, papillary, clear cell, and tanycytic variants, and grade iii anaplastic 14. We present an unusual supratentorial subcortical tanycytic ependymoma in a 17year old male. Cerebellar ependymoma with overlapping features of clearcell. Ependymal tumors classification subependymoma 93831 who grade i myxopapillary ependymoma 93941 who grade i ependymoma papillary ependymoma 93933 who grade ii clear cell ependymoma 939 who grade ii tanycytic ependymoma 939 ependymoma rela fusion positive 93963 anaplastic ependymoma 93923 who grade iii. To best of our knowledge, the present pae nt is the. The positive samples were usually hypercellular, with cohesive epithelioid cells. 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. Among these 32 cases were 2 subependymomas, 19 ependymomas, and 11 anaplastic ependymomas. What is ependymomatypescausessymptomstreatmentprognosis. 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. Tanycytic ependymoma has slightly better prognosis than other ependymoma subtypes.
Our patient had a cpa mass that was a variant of ependymoma known as tanycytic ependymoma that mimicked vestibular schwannoma. Anaplastic ependymoma genetic and rare diseases information. Of the 8 patients with positive andor suspicious diagnoses, 5 ependymomas exhibited anaplastic features and 1 tumor was a myxopapillary ependymoma. For language access assistance, contact the ncats public information officer.
Diagnostic imaging demonstrated a right subcortical pa rietooccipital heterogeous expansive lesion, with growth. Ependymoma is a rare type of brain tumour called a glioma. The spinal cord is made up of bundles of nerve fibers that connect the brain with nerves in most parts of the body. View enhanced pdf access article on wiley online library html view. A place to tell your story so others can read and find hope. There was no family history and hereditary syndrome. Seventeen of them in the spinal cord almost always intramedullary, only three cases were extramedullary. 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. To date, a number of tanycytic ependymomas have been described in both the cerebrum and spinal cord. Tanycytic ependymomas are histological variants of ependymomas, usually found in the spinal cord 1.
Tanycytic ependymoma is an uncommon fibrillar variant of ependymoma characterized by streams of piloid, or hairlike, cells having ependymal nuclei. Tanycytic ependymoma as the glial component of a cerebral ganglioglioma has also been described. Ependymoma cells are known to occasionally exfoliate into. Common symptoms include headaches and feeling or being sick. Anaplastic ependymoma hitskk87 and hitskk92 19871992, 19921997 hitskk87. Symptoms of ependymomas depend on whether the tumour is in the brain or the spinal cord. Senile tanycytic ependymoma that arises from the spinal cord is extremely rare. Tanycitic variant has been recently characterized as a variant of ependymoma, since. If you have problems viewing pdf files, download the latest version of adobe reader. Case report senile tanycytic ependymoma of the spinal cord. Papillary, clear cell, and tanycytic ependymoma are morphological variants without clinical significance beyond that of classical morphological.
Tanycytic ependymoma has been recently classified as a variant of ependymoma by the who. Major histologic variants of ependymoma include cellular, papillary, tanycytic, and clear cell types, all of which are well described. Mar 07, 2018 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. Clear cell ependymoma displays an oligodendrogliallike appearance with perinuclear halos. They start from ependymal cells in the brain or spinal cord. If there is a high index of suspicion for ependymoma then the entire neuroaxis, meaning the brain and entire spinal cord, should be. They do not arise from tanycytes which are located primarily in the floor of the third ventricle. Sir, tanycytic ependymoma is an uncommon fibrillary variant of ependymoma, which was first described in 1978 by friede and pollack. Who classification of cns tumours radiology reference. Know the causes, symptoms, treatment, prognosis of ependymoma.
Keywords ependymoma, pediatric tumor, prognostic factors, morbidity, secondlook surgery disease name ependymoma definition. 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. The part of the brain that is affected depends on where the ependymoma forms. Neurological imaging suggested hemangioblastoma hb. The 5year survival rate tells you what percent of children live at least 5 years after the cancer is found. Mri scans can be very useful and can support but not prove the diagnosis of ependymoma, especially when the tumor is in a common anatomical location.
Tanycytic ependymoma is a rare variant of ependymoma and treatment guidelines are not established. Molecular characterization of histopathological ependymoma. Furthermore, there are even rarer variants of ependymomas that can. Cerebellar ependymoma with overlapping features of clear. Microscopically, this variant consists of highly peomorphic cells with nuclear atypia. Case presentation clinical history a 62yearold chinese woman was admitted to our hos. Magnetic resonance imaging showed intramedullary cystic lesion, in the cervical spine. Ependymoma introduction any tumor that arises from the glial cells in the brain is called a glioma. An ependymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Tanycytic ependymoma the rarest form of grade ii ependymoma. Ependymoma occurs most often in young children, accounting for about 5% of all childhood brain cancers. A case report with summary of prior published cases priyanka maity chaudhuri 1, debashis chakrabarty 1, shubhamitra chaudhuri 2.
Supratentorial tanycytic ependymoma in an adult male. Tanycytic ependymomas usually carry better prognosis as compared to other variant of ependymomas and astrocytomas. It has a unique histology emphasized by the inconspicuous ependymal pattern of cells and close resemblance to schwannoma and astrocytoma. We present an unusual supratentorial subcortical tanycytic ependymoma in a 17year old male presenting with seizures. This uncommon ependymoma variant is composed of elongated, spindleshaped cells with thin eosinophilic fibrillary processes see the following image. Only two other cases of subcortical tanycytic ependymoma have been reported. Tanycytic ependymoma is a rare form of ependymoma that usually arises in the intramedullary spine. Ependymoma brain and spinal cord tumours cancer research uk. Childhood ependymoma is a disease in which malignant cancer cells form in the tissues of the brain and 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.
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. If your baby has an ependymoma, one of the first symptoms you may notice is that he has a larger than usual head. Usually, in pediatric cases the location is intracranial, while in adults it is spinal. Tanycytic ependymoma is a rare fibrillary variant of ependymoma with a predilection for the spinal cord. Central nervous system tumors with ependymal features. We present a case of a supratentorial tanycytic ependymoma in an adult male. Ependymoma, giant cell, intramedullary tumors, spinal tumor, tanycytic key message. Tanycytic ependymoma is a rare subtype of ependymoma that most commonly occurs in the brain. Histologically the tumor was consisted of markedly elongated spindleshaped.
Some studies have concluded that prospective research should be. Review article ependymoma diagnosis and treatment progress. Glial cells provide support and protection for the nerve cells, or neurons, in the brain. These findings led to the diagnosis of ependymoma, likely the tanycytic type who grade ii. The who classification of cns tumours is the most widely accepted system for classifying cns tumours and was based on the histological characteristics of the tumour.
The who classification of tumors of the nervous system oxford. Histological variants are the cellular, papillary, clear cell and tanycytic subtype louis 2007. The tumor consisted of a compact fascicular proliferation of spindle cells having long bipolar cytoplasmic processes, and immunohistochemical and ultrastructural studies demonstrated the ependymal features of neoplastic cells. Clear cell ependymomadisplays an oligodendrogliallike appearance with perinuclear halos. The present case provides further information about the clinicopathology of tanycytic ependymoma with details of the immunohistochemical, ultrastructural and. This report documents a 41yearold woman underwent surgical treatment for a cervical intramedullary tumor. Ependymoma genetic and rare diseases information center. Discussion in 1978, friede and pollak first described tanycytic ependymoma as a variant of ependymoma, being relatively common in the spinal cord. Request pdf supratentorial tanycytic ependymoma tanycytic ependymoma is a rare fibrillary variant of ependymoma with a predilection for the spinal cord. Diagnosis as a world health organization who grade ii tanycytic ependymoma was supported by its appearance on evaluation of the permanent.
Rare ependymoma variants include lipomatous, vacuolated, melanotic, and giant cell types, ependymomas with neuropillike islands, and ependymomas with cartilaginous or osseous differentiation. Supratentorial extraventricular ependymal neoplasms shuangshoti. The brain controls vital functions such as memory and learning, emotion, and the senses hearing, sight, smell, taste, and touch. Tanycytic ependymoma is the rarest variant of ependymoma and occurs primarily in the spinal cord. Their name reflects the morphological similarlity of these tumor cells to the tanycytes. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Computed tomography and magnetic resonance imaging revealed a mass in the cerebellum. Giant cellrich tanycytic ependymoma as intramedullary. Ependymomas arise from ependymal cells that line the ventricles of the brain and the center of the spinal cord. Imaging and histology of clearcell ependymoma and cerebellumbased hemangioblastoma are similar. Microscopic features of higher grade of malignancy are high mitotic activity, florid microvascular proliferation andor pseudopalisading necrosis.
Papillary ependymoma forms linear, epitheliallike surfaces along cerebrospinal fluid exposures. Tanycytic ependymoma is a rare subtybe of ependymoma with a predilection for the spinal cord and intracranial tanycytic ependymoma is thus extremely rare. Jan 05, 2018 tanycytic ependymoma has a predilection for 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. Twentyfive cases have been reported including the authors case. Pdf tanycytic ependymoma is a rare form of ependymoma that usually. 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. Ependymoma is a comparatively rare type of tumor of the central nervous system cns including, the brain and the spinal cord. Among classic ependymomas, the who defines rare histological variants, i. Cerebrospinal fluid cytology in patients with ependymoma.
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. Ijo the tanycytic variant of ependymoma has a piloid, streaming cellular appearance usually not radially oriented around a fibrovascular core, but it can have occasional pseu dorosette formation. Newly diagnosed intracranial or spinal ependymoma all who grades including ependymoma variants. Here we report a case of tanycytic ependymoma occurring at the region of filum terminale in a 44year male who presented with low backache, bilateral lower limb weakness and urinary incontinence. Ependymomas of the anaplastic variety are the most aggressive 15,16. This variant is preferentially located in the supratentorial compartment of the brain. The cause of most childhood brain tumors is unknown.