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==Overview==
==Overview==
Ependymoma may be classified according to the WHO into 3 grades: grades I, II, and III.<ref name=radio>.Ependymomas Dr Bruno Di Muzio and Dr Frank Gaillard Gold Supporter since June 24, 2015">. Radiopaedia.org 2015.http://radiopaedia.org/articles/ependymoma</ref>
Ependymoma may be classified into several subtypes based on [[WHO]] classification (grade I, II, III) and the site of origin.
 
==Classification==
==Classification==


In the most recent World Health Organization (WHO) classification of brain tumors, ependymal tumors are classified into the following four main subtypes:<ref name=Luois>Louis DN, Ohgaki H, Wiestler OD, et al., eds.: WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon, France: IARC Press, 2007.</ref>
*In the most recent World Health Organization (WHO) classification of brain tumors, ependymal tumors are classified into the following four main subtypes:<ref name=Luois>Louis DN, Ohgaki H, Wiestler OD, et al., eds.: WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon, France: IARC Press, 2007.</ref><ref name=Cancergove> Eoendymoma. http://www.cancer.gov/types/brain/hp/child-ependymoma-treatment-pdq#section/_35 URL Accessed on 10 6 2015.</ref>


Subependymoma (WHO Grade I): A subependymoma is a slow-growing neoplasm, typically attached to the ventricle wall and is composed of glial tumor cell clusters embedded in a fibrillary matrix.
:*Subependymoma (WHO grade I): a subependymoma is a slow-growing neoplasm, usually attached to the ventricle wall and is composed of [[Glial cell|glial tumor cell clusters]] ingrained in a [[matrix|fibrillary matrix]].<ref name="pmid25973126">{{cite journal| author=Huang Y| title=Subependymoma with extensive microcystic transformation: a case report. | journal=Int J Clin Exp Pathol | year= 2015 | volume= 8 | issue= 2 | pages= 2191-4 | pmid=25973126 | doi= | pmc=4396238 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25973126  }} </ref>


The true incidence of subependymomas (WHO Grade I) is difficult to determine. These tumors are frequently asymptomatic and may be found incidentally at autopsy. Subependymomas probably comprise less than 5% of all ependymal tumors.
:*Myxopapillary ependymoma (WHO grade I): a myxopapillary ependymoma arises almost exclusively in the location of the conus medullaris, cauda equina, and filum terminale of the spinal cord and is characterized histologically by tumor cells arranged in a papillary manner around vascularized myxoid stromal cores.<ref name="pmid25301811">{{cite journal| author=Weber DC, Wang Y, Miller R, Villà S, Zaucha R, Pica A et al.| title=Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network. | journal=Neuro Oncol | year= 2015 | volume= 17 | issue= 4 | pages= 588-95 | pmid=25301811 | doi=10.1093/neuonc/nou293 | pmc=4483075 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25301811  }} </ref>


Myxopapillary ependymoma (WHO Grade I): A myxopapillary ependymoma arises almost exclusively in the location of the conus medullaris, cauda equina, and filum terminale of the spinal cord and is characterized histologically by tumor cells arranged in a papillary manner around vascularized myxoid stromal cores.
:*Ependymoma (WHO grade II): the ependymoma, which is considered a grade II neoplasm originating from the walls of the ventricles or from the spinal canal, is composed of neoplastic ependymal cells. Ependymomas are subdivided, based on [[histological]] findings, into the following four subtypes<ref name="pmid16794247">{{cite journal| author=Grier JT, Batchelor T| title=Low-grade gliomas in adults. | journal=Oncologist | year= 2006 | volume= 11 | issue= 6 | pages= 681-93 | pmid=16794247 | doi=10.1634/theoncologist.11-6-681 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16794247  }} </ref>:
Ependymoma (WHO Grade II): The ependymoma, which is considered a Grade II neoplasm originating from the walls of the ventricles or from the spinal canal, is composed of neoplastic ependymal cells. Ependymomas are subdivided, based on histological findings, into the following four subtypes:


Cellular ependymoma—the most common subtype; this subtype usually demonstrates significant cellularity without an increase in mitotic activity.
::*[[Cellular]] ependymoma: the most common subtype, this subtype usually demonstrates significant cellularity without an increase in mitotic activity<ref name="pmid27022130">{{cite journal| author=Wu J, Armstrong TS, Gilbert MR| title=Biology and management of ependymomas. | journal=Neuro Oncol | year= 2016 | volume= 18 | issue= 7 | pages= 902-13 | pmid=27022130 | doi=10.1093/neuonc/now016 | pmc=4896548 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27022130  }} </ref>.
Papillary ependymoma—forms linear, epithelial-like surfaces along cerebrospinal fluid exposures.
::*[[Papillary]] ependymoma: forms linear, [[epithelial|epithelial-like]] surfaces along [[cerebrospinal fluid]].
Clear cell ependymoma—displays an oligodendroglial-like appearance with perinuclear halos; this variant is preferentially located in the supratentorial compartment of the brain.
::*[[Clear cell]] ependymoma: displays an [[oligodendroglia|oligodendroglial-like]] appearance with perinuclear halos, this variant is preferentially located in the [[supratentorial]] compartment of the [[brain]]<ref name="pmid22102956">{{cite journal| author=Lee BH, Kwon JT, Park YS| title=Supratentorial clear cell ependymoma mimicking oligodendroglioma : case report and review of the literature. | journal=J Korean Neurosurg Soc | year= 2011 | volume= 50 | issue= 3 | pages= 240-3 | pmid=22102956 | doi=10.3340/jkns.2011.50.3.240 | pmc=3218185 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22102956  }} </ref>.
Tanycytic ependymoma—the rarest form of Grade II ependymoma; this subtype is most commonly found in the spinal cord; tumor cells are arranged in fascicles of variable width and cell density and are poorly intertwined.
::*Tanycytic ependymoma: the rarest form of grade II ependymoma, this subtype is most commonly found in the spinal cord; tumor cells are arranged in fascicles of variable width and cell density and are poorly intertwined<ref name="pmid26755489">{{cite journal| author=Tomek M, Jayajothi A, Brandner S, Jaunmuktane Z, Lee CH, Davagnanam I| title=Imaging features of spinal tanycytic ependymoma. | journal=Neuroradiol J | year= 2016 | volume= 29 | issue= 1 | pages= 61-5 | pmid=26755489 | doi=10.1177/1971400915621322 | pmc=4978340 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26755489  }} </ref>.
 
:*[[Anaplastic]] ependymoma (WHO grade III): also known as malignant ependymoma. An anaplastic ependymoma is considered a malignant [[glioma]] of ependymal differentiation and, compared with the grade II ependymomas, shows increased cellularity and increased mitotic activity. It is often associated with [[microvascular]] proliferation and [[necrosis]]<ref name="pmid26251794">{{cite journal| author=Sarıkafa Ş, Çelik SE, Yarikkaya E, Sayılgan A| title=Malignant Transformation of Grade II Ependymoma in a 2-Year-Old Child: Case Report. | journal=J Neurol Surg Rep | year= 2015 | volume= 76 | issue= 1 | pages= e151-5 | pmid=26251794 | doi=10.1055/s-0035-1549311 | pmc=4520988 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26251794  }} </ref>.
Anaplastic ependymoma (WHO Grade III): Also known as malignant ependymoma. An anaplastic ependymoma is considered a malignant glioma of ependymal differentiation and, compared with the Grade II ependymomas, shows increased cellularity and increased mitotic activity, often associated with microvascular proliferation and necrosis.==Classification==
*Ependymal tumors are also classified based on their sites of origin into:<ref name=Cancergove> Eoendymoma.http://www.cancer.gov/types/brain/hp/child-ependymoma-treatment-pdq#section/_40 URL Accessed on 10 6 2015.</ref><ref name="pmid20615923">{{cite journal| author=Andreiuolo F, Puget S, Peyre M, Dantas-Barbosa C, Boddaert N, Philippe C et al.| title=Neuronal differentiation distinguishes supratentorial and infratentorial childhood ependymomas. | journal=Neuro Oncol | year= 2010 | volume= 12 | issue= 11 | pages= 1126-34 | pmid=20615923 | doi=10.1093/neuonc/noq074 | pmc=PMC3098029 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20615923  }} </ref>
Ependymoma may be classified according to the WHO into 3 grades:<ref name=radio>.Ependymomas Dr Bruno Di Muzio and Dr Frank Gaillard Gold Supporter since June 24, 2015">. Radiopaedia.org 2015.http://radiopaedia.org/articles/ependymoma</ref>
:*[[Supratentorial]] ependymomas<ref name="pmid26425155">{{cite journal| author=Mangalore S, Aryan S, Prasad C, Santosh V| title=Imaging characteristics of supratentorial ependymomas: Study on a large single institutional cohort with histopathological correlation. | journal=Asian J Neurosurg | year= 2015 | volume= 10 | issue= 4 | pages= 276-81 | pmid=26425155 | doi=10.4103/1793-5482.162702 | pmc=4558802 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26425155  }} </ref>
===WHO Grade I===
:*[[Infratentorial]] ependymomas<ref name="pmid25822933">{{cite journal| author=Mandera M, Makarska J, Sobol G, Musioł K| title=Infratentorial ependymomas--a study of the centre in Katowice. | journal=Childs Nerv Syst | year= 2015 | volume= 31 | issue= 7 | pages= 1089-96 | pmid=25822933 | doi=10.1007/s00381-015-2683-9 | pmc=4493855 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25822933  }} </ref>
*Myxopapillary Ependymomas
:*[[Spinal cord]] ependymomas<ref name="pmid20511182">{{cite journal| author=Armstrong TS, Vera-Bolanos E, Bekele BN, Aldape K, Gilbert MR| title=Adult ependymal tumors: prognosis and the M. D. Anderson Cancer Center experience. | journal=Neuro Oncol | year= 2010 | volume= 12 | issue= 8 | pages= 862-70 | pmid=20511182 | doi=10.1093/neuonc/noq009 | pmc=2940672 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20511182  }} </ref>
*Subependymomas
 
===WHO Grade II===
*[[Cellular]] Ependymoma
*[[Papillary]] Ependymoma
*[[Clear cell]] Ependymoma
 
===Grade III===
*[[Anaplastic]] Ependymoma
*Features include
:*Increased cellularity and increased mitotic activity
:*Microvascular proliferation
:*[[Necrosis]]


==References==
==References==
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]

Overview

Ependymoma may be classified into several subtypes based on WHO classification (grade I, II, III) and the site of origin.

Classification

  • In the most recent World Health Organization (WHO) classification of brain tumors, ependymal tumors are classified into the following four main subtypes:[1][2]
  • Myxopapillary ependymoma (WHO grade I): a myxopapillary ependymoma arises almost exclusively in the location of the conus medullaris, cauda equina, and filum terminale of the spinal cord and is characterized histologically by tumor cells arranged in a papillary manner around vascularized myxoid stromal cores.[4]
  • Ependymoma (WHO grade II): the ependymoma, which is considered a grade II neoplasm originating from the walls of the ventricles or from the spinal canal, is composed of neoplastic ependymal cells. Ependymomas are subdivided, based on histological findings, into the following four subtypes[5]:
  • Cellular ependymoma: the most common subtype, this subtype usually demonstrates significant cellularity without an increase in mitotic activity[6].
  • Papillary ependymoma: forms linear, epithelial-like surfaces along cerebrospinal fluid.
  • Clear cell ependymoma: displays an oligodendroglial-like appearance with perinuclear halos, this variant is preferentially located in the supratentorial compartment of the brain[7].
  • Tanycytic ependymoma: the rarest form of grade II ependymoma, this subtype is most commonly found in the spinal cord; tumor cells are arranged in fascicles of variable width and cell density and are poorly intertwined[8].
  • Anaplastic ependymoma (WHO grade III): also known as malignant ependymoma. An anaplastic ependymoma is considered a malignant glioma of ependymal differentiation and, compared with the grade II ependymomas, shows increased cellularity and increased mitotic activity. It is often associated with microvascular proliferation and necrosis[9].
  • Ependymal tumors are also classified based on their sites of origin into:[2][10]

References

  1. ↑ Louis DN, Ohgaki H, Wiestler OD, et al., eds.: WHO Classification of Tumours of the Central Nervous System. 4th ed. Lyon, France: IARC Press, 2007.
  2. ↑ 2.0 2.1 Eoendymoma. http://www.cancer.gov/types/brain/hp/child-ependymoma-treatment-pdq#section/_35 URL Accessed on 10 6 2015.
  3. ↑ Huang Y (2015). "Subependymoma with extensive microcystic transformation: a case report". Int J Clin Exp Pathol. 8 (2): 2191–4. PMC 4396238. PMID 25973126.
  4. ↑ Weber DC, Wang Y, Miller R, Villà S, Zaucha R, Pica A; et al. (2015). "Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network". Neuro Oncol. 17 (4): 588–95. doi:10.1093/neuonc/nou293. PMC 4483075. PMID 25301811.
  5. ↑ Grier JT, Batchelor T (2006). "Low-grade gliomas in adults". Oncologist. 11 (6): 681–93. doi:10.1634/theoncologist.11-6-681. PMID 16794247.
  6. ↑ Wu J, Armstrong TS, Gilbert MR (2016). "Biology and management of ependymomas". Neuro Oncol. 18 (7): 902–13. doi:10.1093/neuonc/now016. PMC 4896548. PMID 27022130.
  7. ↑ Lee BH, Kwon JT, Park YS (2011). "Supratentorial clear cell ependymoma mimicking oligodendroglioma : case report and review of the literature". J Korean Neurosurg Soc. 50 (3): 240–3. doi:10.3340/jkns.2011.50.3.240. PMC 3218185. PMID 22102956.
  8. ↑ Tomek M, Jayajothi A, Brandner S, Jaunmuktane Z, Lee CH, Davagnanam I (2016). "Imaging features of spinal tanycytic ependymoma". Neuroradiol J. 29 (1): 61–5. doi:10.1177/1971400915621322. PMC 4978340. PMID 26755489.
  9. ↑ Sarıkafa Ş, Çelik SE, Yarikkaya E, Sayılgan A (2015). "Malignant Transformation of Grade II Ependymoma in a 2-Year-Old Child: Case Report". J Neurol Surg Rep. 76 (1): e151–5. doi:10.1055/s-0035-1549311. PMC 4520988. PMID 26251794.
  10. ↑ Andreiuolo F, Puget S, Peyre M, Dantas-Barbosa C, Boddaert N, Philippe C; et al. (2010). "Neuronal differentiation distinguishes supratentorial and infratentorial childhood ependymomas". Neuro Oncol. 12 (11): 1126–34. doi:10.1093/neuonc/noq074. PMC 3098029. PMID 20615923.
  11. ↑ Mangalore S, Aryan S, Prasad C, Santosh V (2015). "Imaging characteristics of supratentorial ependymomas: Study on a large single institutional cohort with histopathological correlation". Asian J Neurosurg. 10 (4): 276–81. doi:10.4103/1793-5482.162702. PMC 4558802. PMID 26425155.
  12. ↑ Mandera M, Makarska J, Sobol G, Musioł K (2015). "Infratentorial ependymomas--a study of the centre in Katowice". Childs Nerv Syst. 31 (7): 1089–96. doi:10.1007/s00381-015-2683-9. PMC 4493855. PMID 25822933.
  13. ↑ Armstrong TS, Vera-Bolanos E, Bekele BN, Aldape K, Gilbert MR (2010). "Adult ependymal tumors: prognosis and the M. D. Anderson Cancer Center experience". Neuro Oncol. 12 (8): 862–70. doi:10.1093/neuonc/noq009. PMC 2940672. PMID 20511182.

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