Ependymoma

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Ependymoma
Ependymoma low intermed mag.jpg
Micrograph of an ependymoma. H&E stain.
Specialty Neuro-oncology
Prognosis Five-year survival rate: 83.9% [1]
Frequency200 new cases each year in the United States [2]

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 floor of the fourth ventricle. Rarely, ependymomas can occur in the pelvic cavity.

Contents

Syringomyelia can be caused by an ependymoma. Ependymomas are also seen with neurofibromatosis type II.

Signs and symptoms

Source: [3]

Morphology

Ependymomas are composed of cells with regular, round to oval nuclei. There is a variably dense fibrillary background. Tumor cells may form gland-like round or elongated structures that resemble the embryologic ependymal canal, with long, delicate processes extending into the lumen; more frequently present are perivascular pseudorosettes in which tumor cells are arranged around vessels with an intervening zone consisting of thin ependymal processes directed toward the wall of the vessel. [4]

It has been suggested that ependymomas are derived from radial glia, despite their name suggesting an ependymal origin. [5]

Ependymoma tumors

Ependymomas make up about 5% of adult intracranial gliomas and up to 10% of childhood tumors of the central nervous system (CNS). Their occurrence seems to peak at age 5 years and then again at age 35. 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 fourth ventricle, situated in the lower back portion of the brain, where they may produce headache, nausea and vomiting by obstructing the flow of cerebrospinal fluid. This obstruction may also cause hydrocephalus. They may also arise in the spinal cord, conus medullaris and supratentorial locations. [6] Other symptoms can include (but are not limited to): loss of appetite, difficulty sleeping, temporary inability to distinguish colors, uncontrollable twitching, seeing vertical or horizontal lines when in bright light, and temporary memory loss. It should be remembered that these symptoms also are prevalent in many other illnesses not associated with ependymoma.[ citation needed ]

About 10% of ependymomas are benign myxopapillary ependymoma (MPE). [7] MPE is a localized and slow-growing low-grade tumor, which originates almost exclusively from the lumbosacral nervous tissue of young patients. [7] On the other hand, it is the most common tumor of the lumbosacral canal comprising about 90% of all tumoral lesions in this region. [8]

Although some ependymomas are of a more anaplastic and malignant type, most of them are not anaplastic. Well-differentiated ependymomas are usually treated with surgery. For other ependymomas, total surgical removal is the preferred treatment in addition to radiation therapy. The malignant (anaplastic) varieties of this tumor, malignant ependymoma and the ependymoblastoma, are treated similarly to medulloblastoma but the prognosis is much less favorable. Malignant ependymomas may be treated with a combination of radiation therapy and chemotherapy. Ependymoblastomas, which occur in infants and children younger than 5 years of age, may spread through the cerebrospinal fluid and usually require radiation therapy. The subependymoma, a variant of the ependymoma, is apt to arise in the fourth ventricle but may occur in the septum pellucidum and the cervical spinal cord. It usually affects people over 40 years of age and more often affects men than women. [9]

Extraspinal ependymoma (EEP), also known as extradural ependymoma, may be an unusual form of teratoma [10] or may be confused with a sacrococcygeal teratoma. [11]

Treatment

Guidelines for initial management for ependymoma are maximum surgical resection followed by radiation. [12] Chemotherapy is of limited use and reserved for special cases including young children and those with tumor present after resection. Prophylactic craniospinal irradiation is of variable use and is a source of controversy given that most recurrence occurs at the site of resection and therefore is of debatable efficacy. [13] [12] Confirmation of cerebrospinal infiltration warrants more expansive radiation fields. [14]

Prognosis of recurrence is poor and often indicates palliative care to manage symptoms. [15]

Related Research Articles

<span class="mw-page-title-main">Brain tumor</span> Neoplasm in the brain

A brain tumor occurs when abnormal cells form within the brain. There are two main types of tumors: malignant (cancerous) 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.

<span class="mw-page-title-main">Teratoma</span> Type of germ cell tumor

A teratoma is a tumor made up of several types of tissue, such as hair, muscle, teeth, or bone. Teratomata typically form in the tailbone, ovary, or testicle.

Spinal tumors are neoplasms located in either the vertebral column or the spinal cord. There are three main types of spinal tumors classified based on their location: extradural and intradural. Extradural tumors are located outside the dura mater lining and are most commonly metastatic. Intradural tumors are located inside the dura mater lining and are further subdivided into intramedullary and extramedullary tumors. Intradural-intramedullary tumors are located within the dura and spinal cord parenchyma, while intradural-extramedullary tumors are located within the dura but outside the spinal cord parenchyma. The most common presenting symptom of spinal tumors is nocturnal back pain. Other common symptoms include muscle weakness, sensory loss, and difficulty walking. Loss of bowel and bladder control may occur during the later stages of the disease.

<span class="mw-page-title-main">Meningioma</span> Tumor of the membranes surrounding the brain and spinal cord (meninges)

Meningioma, also known as meningeal tumor, is typically a slow-growing tumor that forms from the meninges, the membranous layers surrounding the brain and spinal cord. Symptoms depend on the location and occur as a result of the tumor pressing on nearby tissue. Many cases never produce symptoms. Occasionally seizures, dementia, trouble talking, vision problems, one sided weakness, or loss of bladder control may occur.

<span class="mw-page-title-main">Germ cell tumor</span> Medical condition

Germ cell tumor (GCT) is a neoplasm derived from the primordial germ cells. Germ-cell tumors can be cancerous or benign. Germ cells normally occur inside the gonads. GCTs that originate outside the gonads may be birth defects resulting from errors during development of the embryo.

<span class="mw-page-title-main">Ependyma</span> Lining of the ventricular system of the brain

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.

<span class="mw-page-title-main">Central canal</span> Cerebrospinal fluid-filled space around the spinal cord

The central canal is the cerebrospinal fluid-filled space that runs through the spinal cord. The central canal lies below and is connected to the ventricular system of the brain, from which it receives cerebrospinal fluid, and shares the same ependymal lining. The central canal helps to transport nutrients to the spinal cord as well as protect it by cushioning the impact of a force when the spine is affected.

Sacrococcygeal teratoma (SCT) is a type of tumor known as a teratoma that develops at the base of the coccyx (tailbone) and is thought to be primarily derived from remnants of the primitive streak. Sacrococcygeal teratomas are benign 75% of the time, malignant 12% of the time, and the remainder are considered "immature teratomas" that share benign and malignant features. Benign sacrococcygeal teratomas are more likely to develop in younger children who are less than 5 months old, and older children are more likely to develop malignant sacrococcygeal teratomas.

<span class="mw-page-title-main">Ganglioglioma</span> Medical condition

A ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently occurs in the temporal lobes of children and young adults. They are mixed cell tumors containing both neural ganglionic cells and neural glial cell components.

<span class="mw-page-title-main">Spinal cord compression</span> Medical condition

Spinal cord compression is a form of myelopathy in which the spinal cord is compressed. Causes can be bone fragments from a vertebral fracture, a tumor, abscess, ruptured intervertebral disc or other lesion.

<span class="mw-page-title-main">Germinoma</span> Medical condition

A germinoma is a type of germ-cell tumor, which is not differentiated upon examination. It may be benign or malignant.

<span class="mw-page-title-main">Atypical teratoid rhabdoid tumor</span> Medical condition

An atypical teratoid rhabdoid tumor (AT/RT) is a rare tumor usually diagnosed in childhood. Although usually a brain tumor, AT/RT can occur anywhere in the central nervous system (CNS), including the spinal cord. About 60% will be in the posterior cranial fossa. One review estimated 52% in the posterior fossa, 39% are supratentorial primitive neuroectodermal tumors (sPNET), 5% are in the pineal, 2% are spinal, and 2% are multifocal.

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">Leptomeningeal cancer</span> Medical condition

Leptomeningeal cancer is a rare complication of cancer in which the disease spreads from the original tumor site to the meninges surrounding the brain and spinal cord. This leads to an inflammatory response, hence the alternative names neoplastic meningitis (NM), malignant meningitis, or carcinomatous meningitis. The term leptomeningeal describes the thin meninges, the arachnoid and the pia mater, between which the cerebrospinal fluid is located. The disorder was originally reported by Eberth in 1870. It is also known as leptomeningeal carcinomatosis, leptomeningeal disease (LMD), leptomeningeal metastasis, meningeal metastasis and meningeal carcinomatosis.

<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).

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/oligodendroglioma 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.

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.

<span class="mw-page-title-main">Astroblastoma</span> Medical condition

Astroblastoma is a rare glial tumor derived from the astroblast, a type of cell that closely resembles spongioblastoma and astrocytes. Astroblastoma cells are most likely found in the supratentorial region of the brain that houses the cerebrum, an area responsible for all voluntary movements in the body. It also occurs significantly in the frontal lobe, parietal lobe, and temporal lobe, areas where movement, language creation, memory perception, and environmental surroundings are expressed. These tumors can be present in major brain areas not associated with the main cerebral hemispheres, including the cerebellum, optic nerve, cauda equina, hypothalamus, and brain stem.

<span class="mw-page-title-main">Central nervous system primitive neuroectodermal tumor</span> Medical condition

A central nervous system primitive neuroectodermal tumor, often abbreviated as PNET, supratentorial PNET, or CNS-PNET, is one of the 3 types of embryonal central nervous system tumors. It is considered an embryonal tumor because it arises from cells partially differentiated or still undifferentiated from birth. Those cells are usually neuroepithelial cells, stem cells destined to turn into glia or neurons. It can occur anywhere within the spinal cord and cerebrum and can have multiple sites of origins, with a high probability of metastasis through cerebrospinal fluid (CSF).

<span class="mw-page-title-main">Anaplastic oligodendroglioma</span> Human brain tumor

Anaplastic oligodendroglioma is a neuroepithelial tumor which is believed to originate from oligodendrocytes, a cell type of the glia. In the World Health Organization (WHO) classification of brain tumors, anaplastic oligodendrogliomas are classified as grade III. In the course of the disease, it can degenerate into highly malignant oligodendroglioma, grade IV. The vast majority of oligodendrogliomas occur sporadically, without a confirmed cause and without inheritance within a family.

References

  1. ↑ "Ependymoma Diagnosis and Treatment". National Cancer Institute. 17 September 2018. Retrieved March 8, 2023.
  2. ↑ "Ependymoma". St. Jude Children's Research Hospital . Retrieved March 8, 2023.
  3. ↑ PRITE 2010 Part II q.13
  4. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). "Ch. 28 The central nervous system". Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. ISBN   0-7216-0187-1.[ page needed ]
  5. ↑ Poppleton H, Gilbertson RJ (January 2007). "Stem cells of ependymoma". British Journal of Cancer. 96 (1): 6–10. doi:10.1038/sj.bjc.6603519. PMC   2360214 . PMID   17179988.
  6. ↑ Goel A, Gaillard F. "Ependymoma". Radiopaedia.org. Retrieved 12 September 2014.
  7. 1 2 Mehrjardi MZ, Mirzaei S, Haghighatkhah HR (2017). "The many faces of primary cauda equina myxopapillary ependymoma: Clinicoradiological manifestations of two cases and review of the literature". Romanian Neurosurgery. 31 (3): 385–90. doi: 10.1515/romneu-2017-0062 .
  8. ↑ Duong LM, McCarthy BJ, McLendon RE, Dolecek TA, Kruchko C, Douglas LL, Ajani UA (September 2012). "Descriptive epidemiology of malignant and nonmalignant primary spinal cord, spinal meninges, and cauda equina tumors, United States, 2004-2007". Cancer. 118 (17): 4220–4227. doi:10.1002/cncr.27390. PMC   4484585 . PMID   22907705.
  9. ↑ Pan E, Prados MD (2003). "Ependymoma". Holland-Frei Cancer Medicine (6th ed.). BC Decker.
  10. ↑ Aktuğ T, Hakgüder G, Sarioğlu S, Akgür FM, Olguner M, Pabuçcuoğlu U (March 2000). "Sacrococcygeal extraspinal ependymomas: the role of coccygectomy". Journal of Pediatric Surgery. 35 (3): 515–518. doi:10.1016/S0022-3468(00)90228-8. PMID   10726703.
  11. ↑ Hany MA, Bouvier R, Ranchère D, Bergeron C, Schell M, Chappuis JP, et al. (2009). "Case report: a preterm infant with an extradural myxopapillary ependymoma component of a teratoma and high levels of alpha-fetoprotein". Pediatric Hematology and Oncology. 15 (5): 437–441. doi:10.3109/08880019809016573. PMID   9783311.
  12. 1 2 Reni M (2003). "Guidelines for the treatment of adult intra-cranial grade II-III ependymal tumours". Forum. 13 (1): 90–98. PMID   14732890.
  13. ↑ Merchant TE, Fouladi M (December 2005). "Ependymoma: new therapeutic approaches including radiation and chemotherapy". Journal of Neuro-Oncology. 75 (3): 287–299. doi:10.1007/s11060-005-6753-9. PMID   16195801. S2CID   37438304.
  14. ↑ "Ependymoma". The Lecturio Medical Concept Library. Retrieved 19 July 2021.
  15. ↑ Merchant TE, Boop FA, Kun LE, Sanford RA (May 2008). "A retrospective study of surgery and reirradiation for recurrent ependymoma". International Journal of Radiation Oncology, Biology, Physics. 71 (1): 87–97. doi: 10.1016/j.ijrobp.2007.09.037 . PMID   18406885.
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