Subependymoma

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Subependymoma
Subependymoma - high mag.jpg
Micrograph of a subependymoma showing the characteristic clustering of nuclei. H&E stain.
Specialty Neoplasms, Neuro-oncology

A subependymoma is a type of brain tumor; specifically, it is a rare form of ependymal tumor. [1] They are usually in middle aged people. Earlier, they were called subependymal astrocytomas. [2]

Contents

The prognosis for a subependymoma is better than for most ependymal tumors, [3] and it is considered a grade I tumor in the World Health Organization (WHO) classification.

They are classically found within the fourth ventricle, typically have a well demarcated interface to normal tissue and do not usually extend into the brain parenchyma, like ependymomas often do. [4]

Symptoms and signs

Subependymomas of the fourth ventricle, extending into the cerebellopontine angle via the foramen of Luschka, right side of illustration. Subependymoma.jpg
Subependymomas of the fourth ventricle, extending into the cerebellopontine angle via the foramen of Luschka, right side of illustration.

Patients are often asymptomatic, and are incidentally diagnosed. Larger tumours are often with increased intracranial pressure. [2]

Pathology

These tumours are small, no more than two centimeters across, coming from the ependyma. The best way to distinguish it from a subependymal giant cell astrocytoma is the size. [2]

Diagnosis

The diagnosis is based on tissue, e.g. a biopsy. Histologically subependymomas consistent of microcystic spaces and bland appearing cells without appreciable nuclear atypia or mitoses. The nuclei tend to form clusters.[ citation needed ]

On a CT, it often shows a less dense to equally dense mass. If it is big, it may have parts that are cystic or calcific. [2] In 50-60% of cases, the tumor is in the fourth ventricle, while the second most common (30-40% of cases) location is the side ventricles. It is rare for it to be in the third ventricle or the central canal of the spinal cord. [2]

Treatment

Asymptomatic cases may only need watchful waiting. If symptomatic, it can be surgically removed, and partial removal also carries an excellent prognosis. [2]

Prognosis

The outlook of a cure is extremely favorable. [2]

Related Research Articles

Brain tumor Neoplasm in the brain

A brain tumor occurs when abnormal cells form within the brain. There are two main types of tumors: malignant tumors and benign (non-cancerous) tumors. These can be further classified as primary tumors, which start within the brain, and secondary tumors, which most commonly have spread from tumors located outside the brain, known as brain metastasis tumors. All types of brain tumors may produce symptoms that vary depending on the size of the tumor and the part of the brain that is involved. Where symptoms exist, they may include headaches, seizures, problems with vision, vomiting and mental changes. Other symptoms may include difficulty walking, speaking, with sensations, or unconsciousness.

Choroid plexus

The choroid plexus, or plica choroidea, is a plexus of cells that arises from the tela choroidea in each of the ventricles of the brain. The choroid plexus produces most of the cerebrospinal fluid (CSF) of the central nervous system. CSF is produced and secreted by the regions of the choroid plexus. The choroid plexus consists of modified ependymal cells surrounding a core of capillaries and loose connective tissue.

Tuberous sclerosis Genetic condition causing non-cancerous tumours

Tuberous sclerosis complex (TSC) is a rare multisystem autosomal dominant genetic disease that causes non-cancerous tumours to grow in the brain and on other vital organs such as the kidneys, heart, liver, eyes, lungs and skin. A combination of symptoms may include seizures, intellectual disability, developmental delay, behavioral problems, skin abnormalities, lung disease, and kidney disease.

Glioma Tumour of the glial cells of the brain or spine

A glioma is a type of tumor that starts in the glial cells of the brain or the spine. Gliomas comprise about 30 percent of all brain tumors and central nervous system tumours, and 80 percent of all malignant brain tumours.

Ependymoma Medical condition

An ependymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Usually, in pediatric cases the location is intracranial, while in adults it is spinal. The common location of intracranial ependymomas is the fourth ventricle. Rarely, ependymomas can occur in the pelvic cavity.

Astrocytoma Medical condition

Astrocytomas are a type of brain tumor. They originate in a particular kind of glial cells, star-shaped brain cells in the cerebrum called astrocytes. This type of tumor does not usually spread outside the brain and spinal cord and it does not usually affect other organs. Astrocytomas are the most common glioma and can occur in most parts of the brain and occasionally in the spinal cord.

Ependyma

The ependyma is the thin neuroepithelial lining of the ventricular system of the brain and the central canal of the spinal cord. The ependyma is one of the four types of neuroglia in the central nervous system (CNS). It is involved in the production of cerebrospinal fluid (CSF), and is shown to serve as a reservoir for neuroregeneration.

Calcification Calcification in bones

Calcification is the accumulation of calcium salts in a body tissue. It normally occurs in the formation of bone, but calcium can be deposited abnormally in soft tissue, causing it to harden. Calcifications may be classified on whether there is mineral balance or not, and the location of the calcification. Calcification may also refer to the processes of normal mineral deposition in biological systems, such as the formation of stromatolites or mollusc shells.

Pilocytic astrocytoma Medical condition

Pilocytic astrocytoma is a brain tumor that occurs most commonly in children and young adults. They usually arise in the cerebellum, near the brainstem, in the hypothalamic region, or the optic chiasm, but they may occur in any area where astrocytes are present, including the cerebral hemispheres and the spinal cord. These tumors are usually slow growing and benign, corresponding to WHO malignancy grade 1.

Medulloblastoma Most common type of primary brain cancer in children

Medulloblastoma is a common type of primary brain cancer in children. It originates in the part of the brain that is towards the back and the bottom, on the floor of the skull, in the cerebellum, or posterior fossa.

The subependymal zone (SEZ) is a cell layer below the ependyma in the lateral ventricles of the brain. It is an adult version of the embryonic forebrain germinal zone. This region contains adult neural stem cells, also called neuroepithelial cells, which have the potential to generate new neurons and glial cells. The generation of neurons and glial cells from neuroepithelial cells occurs via neurogenesis and gliogenesis, respectively. In adults, the subependymal zone is also called the subventricular zone, as the ependymal cell layer forms the boundary between the fluid-filled ventricular space and the walls of the lateral ventricles.

Glial tumor is a general term for numerous tumors of the central nervous system, including astrocytomas, ependymal tumors, Oligodendroglioma, and primitive neuroectodermal tumors. The World Health Organization (WHO) classifies tumors into different categories according to severity and recurrence.The first tumor classified as grade I is called pilocytic astrocytoma and it is most commonly observed in children rather than adults. The next tumor is never common in the Dns called diffuse astrocytoma and it is considered a grade II, they are benign, or noncancerous, but can become malignant, meaning cancerous, as the tumor progresses. Grades III and grade IV are considered malignant astrocytomas. Anaplastic astrocytomas are considered by the WHO to be a grade III astrocytoma and Glioblastoma is a grade IV both are referred to high-grade glial tumors.

Choroid plexus carcinoma Medical condition

A choroid plexus carcinoma is a type of choroid plexus tumor that affects the choroid plexus of the brain. It is considered the worst of the three grades of chord plexus tumors, having a much poorer prognosis than choroid atypical plexus papilloma and choroid plexus papilloma. The disease creates lesions in the brain and increases cerebrospinal fluid volume, resulting in hydrocephalus.

Central nervous system cyst Medical condition

A central nervous system cyst is a type of cyst that presents and affects part of the central nervous system (CNS). They are usually benign and filled with either cerebrospinal fluid, blood, or tumor cells. CNS cysts are classified into two categories: cysts that originate from non-central nervous system tissue, migrate to, and form on a portion of the CNS, and cysts that originate within central nervous system tissue itself. Within these two categories, there are many types of CNS cysts that have been identified from previous studies.

WHO classification of tumours of the central nervous system

The following is a simplified (deprecated) version of the 2021 WHO classification of the tumours of the central nervous system. Currently, as of 2021, clinicians are using the WHO grade 5th edition, which incorporates recent advances in molecular pathology.

Neuro-oncology is the study of brain and spinal cord neoplasms, many of which are very dangerous and life-threatening. Among the malignant brain cancers, gliomas of the brainstem and pons, glioblastoma multiforme, and high-grade astrocytoma are among the worst. In these cases, untreated survival usually amounts to only a few months, and survival with current radiation and chemotherapy treatments may extend that time from around a year to a year and a half, possibly two or more, depending on the patient's condition, immune function, treatments used, and the specific type of malignant brain neoplasm. Surgery may in some cases be curative, but, as a general rule, malignant brain cancers tend to regenerate and emerge from remission easily, especially highly malignant cases. In such cases, the goal is to excise as much of the mass and as much of the tumor margin as possible without endangering vital functions or other important cognitive abilities. The Journal of Neuro-Oncology is the longest continuously published journal in the field and serves as a leading reference to those practicing in the area of neuro-oncology.

Fibrillary astrocytoma Type of brain tumor

Fibrillary astrocytomas are a group of primary slow-growing brain tumors that typically occur in adults between the ages of 20 and 50.

Subependymal giant cell astrocytoma Medical condition

Subependymal giant cell astrocytoma is a low-grade astrocytic brain tumor (astrocytoma) that arises within the ventricles of the brain. It is most commonly associated with tuberous sclerosis complex (TSC). Although it is a low-grade tumor, its location can potentially obstruct the ventricles and lead to hydrocephalus.

Pediatric ependymomas are similar in nature to the adult form of ependymoma in that they are thought to arise from radial glial cells lining the ventricular system. However, they differ from adult ependymomas in which genes and chromosomes are most often affected, the region of the brain they are most frequently found in, and the prognosis of the patients. Children with certain hereditary diseases, such as neurofibromatosis type II (NF2), have been found to be more frequently afflicted with this class of tumors, but a firm genetic link remains to be established. Symptoms associated with the development of pediatric ependymomas are varied, much like symptoms for a number of other pediatric brain tumors including vomiting, headache, irritability, lethargy, and changes in gait. Although younger children and children with invasive tumor types generally experience less favorable outcomes, total removal of the tumors is the most conspicuous prognostic factor for both survival and relapse.

Palisade (pathology) Single layer of relatively long cells arranged loosely perpendicular to a perpendicular to a surface and parallel to each other

In histopathology, a palisade is a single layer of relatively long cells, arranged loosely perpendicular to a surface and parallel to each other. A rosette is a palisade in a halo or spoke-and-wheel arrangement, surrounding a central core or hub. A pseudorosette is a perivascular radial arrangement of neoplastic cells around a small blood vessel.

References

  1. Orakcioglu B, Schramm P, Kohlhof P, Aschoff A, Unterberg A, Halatsch ME (January 2009). "Characteristics of thoracolumbar intramedullary subependymomas". Journal of Neurosurgery. Spine. 10 (1): 54–59. doi:10.3171/2008.10.SPI08311. PMID   19119934.
  2. 1 2 3 4 5 6 7 Gaillard, Frank. "Subependymoma | Radiology Reference Article | Radiopaedia.org". radiopaedia.org. Retrieved 2018-04-15.
  3. Prayson RA, Suh JH (April 1999). "Subependymomas: clinicopathologic study of 14 tumors, including comparative MIB-1 immunohistochemical analysis with other ependymal neoplasms". Archives of Pathology & Laboratory Medicine. 123 (4): 306–309. doi:10.5858/1999-123-0306-S. PMID   10320142.
  4. Hoeffel C, Boukobza M, Polivka M, Lot G, Guichard JP, Lafitte F, et al. (1995). "MR manifestations of subependymomas". AJNR. American Journal of Neuroradiology. 16 (10): 2121–2129. PMID   8585504.
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