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Ependymoma of 4.ventricle in MRI.

Ependymoma of 4.ventricle in MRI. Left without, right with contrast-enhancement.

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 ependymoma is the fourth ventricle. Rarely, ependymoma can occur in the pelvic cavity.

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

Symptoms

severe headache
visual loss (due to papilledema)
vomiting
bilateral Babinski sign
drowsiness (after several hours of the above symptoms)
gait change (rotation of feet when walking)
impaction/constipation
back flexibility

^[1]

Morphology

Micrograph of a myxopapillary ependymoma. HPS stain.

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.^[2]

It has been suggested that ependymomas are derived from radial glia.^[3]

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 medularis and supratentorial locations.^[4] 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.

About 10% of ependymomas are benign myxopapillary ependymoma (MPE)^{[citation needed]}. MPE is a localized and slowly growing, low-grade tumor. 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.

Extraspinal ependymoma (EEP), also known as extradural ependymoma, may be an unusual form of teratoma^[5] or may be confused with a sacrococcygeal teratoma.^[6]

References

^ PRITE 2010 Part II q.13
^ 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.
^ Poppleton H, Gilbertson RJ (January 2007). "Stem cells of ependymoma". Br. J. Cancer 96 (1): 6–10. doi:10.1038/sj.bjc.6603519. PMC 2360214. PMID 17179988.
^ Goel, Ayush; Gaillard, Frank. "Ependymoma". Radiopaedia.org. Retrieved 12 September 2014.
^ Aktuğ T, Hakgüder G, Sarioğlu S, Akgür FM, Olguner M, Pabuçcuoğlu U (March 2000). "Sacrococcygeal extraspinal ependymomas: the role of coccygectomy". J. Pediatr. Surg. 35 (3): 515–8. doi:10.1016/S0022-3468(00)90228-8. PMID 10726703.
^ Hany MA, Bouvier R, Ranchère D, Bergeron C, Schell M, Chappuis JP, Philip T, Frappaz D (1998). "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): 437–41. doi:10.3109/08880019809016573. PMID 9783311.

External links

Collaborative Ependymoma Research Network (CERN)
Brain and Spinal Tumors: Hope Through Research (National Institute of Neurological Disorders and Stroke)
Cancer.Net: Ependymoma, Childhood

Images and technical information

Adult Ependymoma Images
Pediatric Ependymoma Images
Ependymoma Topics — MedPix
Ependymoma Guide for Patients, Caregivers and Advocates - CERN Foundation
Ependymoma Information from ABTA (PDF) for Completely Resected, Differentiated, Supratentorial Ependymoma]
St. Jude Ependymoma Information
Cancer Back Up — Ependymoma
Walid MS, Parveen T, Grigorian AA, Robinson J (2007). "Chronic Back Pain: Clinical Quiz". The Internet Journal of Neurosurgery 4 (1).
Spinal Cord Tumor Association

Treatment Information

Treatment Information — from the CERN Foundation
Treatment for Adults
Pediatric Treatment
Ependymoma-Specific Clinical Trials
Ependymoma Outcomes Survey Project

UpToDate Contents

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1. 脳室上衣細胞腫 ependymoma
2. 脊髄腫瘍 spinal cord tumors
3. 神経膠腫の分類 classification of gliomas
4. 小児における中枢神経系腫瘍の疫学 epidemiology of central nervous system tumors in children
5. 小児における中枢神経系腫瘍のマネージメントに関する概要 overview of the management of central nervous system tumors in children

English Journal

Pelvic Ependymoma With Clinical Response to GnRH Analog Therapy: A Case Report With an Overview of Primary Extraneural Ependymomas.

Zhou F1, Song J, Mikolaenko I, Rosenblum M, Shukla PS.
International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists.Int J Gynecol Pathol.2015 Sep;34(5):450-8. doi: 10.1097/PGP.0000000000000156.
Extraneural ependymomas are rare tumors that occur in sacrococcygeal, pelvic and extra pelvic regions. While sacrococcygeal extraneural ependymomas are equally distributed among males and females, pelvic and extra pelvic ependymomas have been exclusively reported in women, mainly of child bearing ag
PMID 26107559

Imaging Changes in Pediatric Intracranial Ependymoma Patients Treated With Proton Beam Radiation Therapy Compared to Intensity Modulated Radiation Therapy.

Gunther JR1, Sato M2, Chintagumpala M2, Ketonen L3, Jones JY4, Allen PK1, Paulino AC5, Okcu MF2, Su JM2, Weinberg J6, Boehling NS1, Khatua S7, Adesina A8, Dauser R9, Whitehead WE9, Mahajan A10.
International journal of radiation oncology, biology, physics.Int J Radiat Oncol Biol Phys.2015 Sep 1;93(1):54-63. doi: 10.1016/j.ijrobp.2015.05.018. Epub 2015 May 16.
PURPOSE: The clinical significance of magnetic resonance imaging (MRI) changes after radiation therapy (RT) in children with ependymoma is not well defined. We compared imaging changes following proton beam radiation therapy (PBRT) to those after photon-based intensity modulated RT (IMRT).METHODS A
PMID 26279024

Ependymoma in adults: Local experience with an uncommon tumour.

Asaid M1, Preece PD2, Rosenthal MA3, Drummond KJ2.
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia.J Clin Neurosci.2015 Sep;22(9):1392-6. doi: 10.1016/j.jocn.2015.03.020. Epub 2015 Jun 18.
This study reviews our tertiary hospital experience in an adult population of ependymoma patients. Ependymomas are uncommon tumours of the central nervous system (CNS) and the literature provides little information to guide management and predict prognosis. The prospectively maintained Australian Co
PMID 26094561

Japanese Journal

第四脳室と橋・延髄の解剖と外科治療(<特集>脳室・脳幹病変の外科治療)

斉藤延人,金太一
脳神経外科ジャーナル 20(6), 438-445, 2011-06-20
第四脳室と橋・延髄下半分の病変には,髄芽腫,脳室上衣腫,星細胞腫,脈絡叢乳頭腫,脳幹海綿状血管腫,血管芽腫,外向発育性グリオーマなどがある.第四脳室を露出するには小脳延髄裂アプローチが有用である.小脳扁桃の裏で延髄に付着している脈絡膜と脈絡紐を切開して小脳扁桃を牽引する方法である.脳幹内部へは,病変が最も表面に近い部分から進入するのが基本である.第四脳室底には正中溝,閂,第四脳室線条,外側陥凹,顔 …
NAID 110008662231

Neural stem cell-like gene expression in a mouse ependymoma cell line transformed by human BK polyomavirus

AIZAWA Takako,HASEGAWA Koichi,OHKUMO Tsuyoshi,HAGA Seiichi,IKEDA Kazuhiko,YOSHIKAWA Kazuaki
Cancer science 102(1), 122-129, 2011-01-10
NAID 10029288727

Related Pictures

★リンクテーブル★

リンク元	「脳腫瘍」「神経膠腫」「上衣腫」「上衣細胞腫」「脳室上衣腫」
拡張検索	「myxopapillary ependymoma」「papillary ependymoma」「anaplastic ependymoma」

「脳腫瘍」

Ependymoma
	Micrograph of an ependymoma. H&E stain.
Classification and external resources
Specialty	Oncology
ICD-10	C71
ICD-9-CM	191.9, 225.0, 237.5
ICD-O	M9391/3-9394/1
DiseasesDB	29452
eMedicine	med/700
MeSH	D004806
	[edit on Wikidata]

　　[★]

英: brain tumor, cerebral tumor
同: 脳新生物 brain neoplasm
関: 頭蓋内腫瘍

概念

分類

原発性脳腫瘍

神経膠腫 glioma

星状細胞腫 astrocytoma

胚腫 germinoma

転移性脳腫瘍

疫学

発生率：10万人対10-15人。罹患率：10万人対46人　→

腫瘍別発生頻度

腫瘍別発生頻度		小児	成人
神経膠腫	33%	星状細胞腫	髄膜腫
髄膜腫	22%	髄芽腫	膠芽腫
下垂体腺腫	15%	頭蓋咽頭腫	下垂体腺腫
神経鞘腫	9%	胚細胞腫	神経鞘腫
頭蓋咽頭腫	5%	上衣腫	転移性脳腫瘍

YN.J.188

部位	種類	小児	成人
頭蓋骨	頭蓋骨腫瘍	○	○
大脳半球	神経膠腫		○
大脳半球	髄膜腫		○
松果体	胚細胞腫	○
小脳半球	星細胞腫	○
小脳半球	血管芽腫		○
小脳虫部	髄芽腫	○
第四脳室	上衣腫	○
鞍上部・視交叉部・下垂体部	頭蓋咽頭腫	○
	視神経膠腫	○
	胚細胞腫	○
	下垂体腺腫		○
	髄膜腫		○
小脳橋角部	聴神経鞘腫		○
脳幹部	神経膠腫	○	○

小児の脳腫瘍

SCN.173

腫瘍：星細胞腫、髄芽腫、頭蓋咽頭腫、胚細胞腫、上衣腫の順に多い。
部位：1歳まではテント上、2-7歳まではテント下、8-15歳まではテント上に多い。

SRA.163

	後発年齢	好発部位
小脳星細胞腫	5～10歳	小脳半球
髄芽腫	5～10歳(男児に多い)	小脳虫部から発生
頭蓋咽頭腫	10～15歳	トルコ鞍上部
上衣腫	10～15,30～40歳	第四脳室、側脳室
髄芽腫	10～30歳	松果体部、トルコ鞍上部
脳幹部膠腫	～15歳	橋
視神経膠腫	～15歳	視神経視交叉