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]

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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.

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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.

<span class="mw-page-title-main">Choroid plexus carcinoma</span> Medical condition

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<span class="mw-page-title-main">WHO classification of tumours of the central nervous system</span>

The WHOclassification of tumours of the central nervous system is a World Health Organization Blue Book that defines, describes and classifies tumours of the central nervous system (CNS).

<span class="mw-page-title-main">Subependymal giant cell astrocytoma</span> 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.

<span class="mw-page-title-main">Angiocentric glioma</span>

Angiocentric glioma (AG) refers to a rare neuroepithelial tumor when the superficial brain malignant cells enclose the brain vessels, commonly found in children and young adults. Initially identified in 2005 by Wang and his team from the University of Texas, AG was classified as Grade I by 2007 WHO Classification of Tumors of the Central Nervous System due to its benign clinical behavior, low proliferation index, and curative properties. AG primarily affects children and young adults at an average initial diagnosis age of 16 years old. Over 85% AG patients experience intractable seizures since childhood, especially partial epilepsy.

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. PMC   8337222 . PMID   8585504.