Difference between revisions of "Subependymoma"

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==Historical Perspective==
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Subependymoma was first discovered by Ilya Mark Scheinker, a Russian physician, in 1945.<ref name="pmid18397339">{{cite journal| author=Kurian KM, Jones DT, Marsden F, Openshaw SW, Pearson DM, Ichimura K et al.| title=Genome-wide analysis of subependymomas shows underlying chromosomal copy number changes involving chromosomes 6, 7, 8 and 14 in a proportion of cases. | journal=Brain Pathol | year= 2008 | volume= 18 | issue= 4 | pages= 469-73 | pmid=18397339 | doi=10.1111/j.1750-3639.2008.00148.x | pmc=2659379 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18397339  }} </ref>
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 +
==Classification==
 +
There is no established system for the [[classification]] of subependymoma.
  
 
==Pathophysiology==
 
==Pathophysiology==
 
===Pathogenesis===
 
===Pathogenesis===
*Subependymoma arises from [[subependymal]] glial cells, although it can also arise from [[Astrocyte|astrocytes]] from the [[subependymal]] plate, [[ependymal cells]], and mixed ependymal and astrocytic cells.<ref name="pmid23382616">{{cite journal| author=Saad AF, Bidiwala SB, Layton KF, Snipes GJ, Opatowsky MJ| title=Fourth ventricular subependymoma presenting as worsening headache. | journal=Proc (Bayl Univ Med Cent) | year= 2013 | volume= 26 | issue= 1 | pages= 52-4 | pmid=23382616 | doi= | pmc=PMC3523772 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23382616  }} </ref><ref name="pmid22747714">{{cite journal| author=Jain A, Amin AG, Jain P, Burger P, Jallo GI, Lim M et al.| title=Subependymoma: clinical features and surgical outcomes. | journal=Neurol Res | year= 2012 | volume= 34 | issue= 7 | pages= 677-84 | pmid=22747714 | doi=10.1179/1743132812Y.0000000064 | pmc=4618470 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22747714  }} </ref>
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Subependymoma arises from [[subependymal]] [[Glial cell|glial cells]], although it can also arise from [[Astrocyte|astrocytes]] from the [[subependymal]] plate, [[ependymal cells]], and mixed ependymal and astrocytic cells.<ref name="pmid23382616">{{cite journal| author=Saad AF, Bidiwala SB, Layton KF, Snipes GJ, Opatowsky MJ| title=Fourth ventricular subependymoma presenting as worsening headache. | journal=Proc (Bayl Univ Med Cent) | year= 2013 | volume= 26 | issue= 1 | pages= 52-4 | pmid=23382616 | doi= | pmc=PMC3523772 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23382616  }} </ref><ref name="pmid22747714">{{cite journal| author=Jain A, Amin AG, Jain P, Burger P, Jallo GI, Lim M et al.| title=Subependymoma: clinical features and surgical outcomes. | journal=Neurol Res | year= 2012 | volume= 34 | issue= 7 | pages= 677-84 | pmid=22747714 | doi=10.1179/1743132812Y.0000000064 | pmc=4618470 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22747714  }} </ref>
  
 
===Gross Pathology===
 
===Gross Pathology===
*Subependymoma is most commonly seen in the [[fourth ventricle]], but can arise anywhere where there is [[ependyma]]. The distribution in the [[ventricular system]] is as follows:<ref name="pathoilogysubepenymoma1">Pathology of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref><ref name="pmid22747714">{{cite journal| author=Jain A, Amin AG, Jain P, Burger P, Jallo GI, Lim M et al.| title=Subependymoma: clinical features and surgical outcomes. | journal=Neurol Res | year= 2012 | volume= 34 | issue= 7 | pages= 677-84 | pmid=22747714 | doi=10.1179/1743132812Y.0000000064 | pmc=4618470 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22747714  }} </ref>
+
Subependymoma is most commonly seen in the [[fourth ventricle]], but it can arise anywhere where there is [[ependyma]]. The distribution in the [[ventricular system]] is as follows:<ref name="pathoilogysubepenymoma1">Pathology of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref><ref name="pmid22747714" />
**[[Fourth ventricle]]: 50-60%
+
 
**[[Lateral ventricles]] (usually frontal horns): 30-40%
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*[[Fourth ventricle]]: 50 - 60%
**[[Third ventricle]]: rare
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*[[Lateral ventricles]] (usually frontal horns): 30 - 40%
**[[Central canal|Central canal of the spinal cord]]: rare
+
*[[Third ventricle]]: rare
*On gross pathology, subependymoma is characterized by a small, white to grey, firm, well circumscribed, solid, [[avascular]] mass attached to the ventricular wall by a narrow [[Pedicles|pedicle]].<ref name="pmid23382616">{{cite journal| author=Saad AF, Bidiwala SB, Layton KF, Snipes GJ, Opatowsky MJ| title=Fourth ventricular subependymoma presenting as worsening headache. | journal=Proc (Bayl Univ Med Cent) | year= 2013 | volume= 26 | issue= 1 | pages= 52-4 | pmid=23382616 | doi= | pmc=PMC3523772 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23382616  }} </ref><ref name="pmid22747714">{{cite journal| author=Jain A, Amin AG, Jain P, Burger P, Jallo GI, Lim M et al.| title=Subependymoma: clinical features and surgical outcomes. | journal=Neurol Res | year= 2012 | volume= 34 | issue= 7 | pages= 677-84 | pmid=22747714 | doi=10.1179/1743132812Y.0000000064 | pmc=4618470 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22747714  }} </ref>
+
*[[Central canal|Central canal of the spinal cord]]: rare
 +
 
 +
*On gross pathology, subependymoma is characterized by a small, white to grey, firm, well circumscribed, solid, [[avascular]] mass attached to the ventricular wall by a narrow [[Pedicles|pedicle]].<ref name="pmid23382616" /><ref name="pmid22747714" />
  
 
===Microscopic Pathology===
 
===Microscopic Pathology===
*On microscopic histopathological analysis, subependymoma is characterized by microcystic spaces and bland appearing cells without appreciable [[atypia|nuclear atypia]] or [[mitoses]]. The nuclei tend to form clusters. No high grade features (mitoses, [[Ki-67 (Biology)|Ki-67]] / [[MIB1|MIBI]] index > 1.5%, necrosis) are present. Loose pseudorosettes are observed.<ref name="pathoilogysubepenymoma1">Pathology of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref>
+
On [[microscopic]] [[Histopathology|histopathological]] [[analysis]], subependymoma is characterized by the following features:<ref name="pathoilogysubepenymoma1" />
 +
 
 +
* Microcystic spaces and bland appearing [[Cell (biology)|cells]] without appreciable [[atypia|nuclear atypia]] or [[mitoses]].  
 +
* The [[Cell nucleus|nuclei]] tend to form clusters.  
 +
* No high grade features ([[Mitosis|mitoses]], [[Ki-67 (Biology)|Ki-67]] / [[MIB1|MIBI]] index > 1.5%, [[necrosis]]) are present.  
 +
* Loose pseudorosettes are observed.
  
 
===Immunohistochemistry===
 
===Immunohistochemistry===
*Subependymoma is demonstrated by positivity to tumor marker such as [[GFAP]].<ref name="pathoilogysubepenymoma1">Pathology of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref>
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Subependymoma is characterized by positive [[tumor marker]] [[GFAP]]. Mixed populations of [[Cell (biology)|cells]] may be variably positive for:<ref name="pathoilogysubepenymoma1" /><ref name="pmid28804038">{{cite journal| author=D'Amico RS, Praver M, Zanazzi GJ, Englander ZK, Sims JS, Samanamud JL et al.| title=Subependymomas Are Low-Grade Heterogeneous Glial Neoplasms Defined by Subventricular Zone Lineage Markers. | journal=World Neurosurg | year= 2017 | volume= 107 | issue=  | pages= 451-463 | pmid=28804038 | doi=10.1016/j.wneu.2017.08.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28804038  }} </ref>
 +
 
 +
*Olig2
 +
*NHERF1
 +
*[[Sox2]]
 +
*[[CD44]]
 +
 
 +
==Causes==
 +
The cause of the development of subependymoma has not been identified.
 +
 
 +
==Differentiating Subependymoma from Other Diseases==
 +
Subependymoma must be differentiated from:<ref name="ddxse1">Intraventricular neoplasms and lesions. Dr Henry Knipe and Dr Vinod G Maller et al. Radiopaedia 2016. http://radiopaedia.org/articles/intraventricular-neoplasms-and-lesions. Accessed on January 12, 2016</ref><ref name="pmid22747714" />
  
==Differentiating Subependymoma from other Diseases==
+
*'''Neoplasms of the ventri­cular wall and septum pellucidum'''
*Subependymoma must be differentiated from:<ref name="ddxse1">Intraventricular neoplasms and lesions. Dr Henry Knipe and Dr Vinod G Maller et al. Radiopaedia 2016. http://radiopaedia.org/articles/intraventricular-neoplasms-and-lesions. Accessed on January 12, 2016</ref><ref name="pmid22747714">{{cite journal| author=Jain A, Amin AG, Jain P, Burger P, Jallo GI, Lim M et al.| title=Subependymoma: clinical features and surgical outcomes. | journal=Neurol Res | year= 2012 | volume= 34 | issue= 7 | pages= 677-84 | pmid=22747714 | doi=10.1179/1743132812Y.0000000064 | pmc=4618470 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22747714  }} </ref>
+
**[[Ependymoma]]
**'''Neoplasms of the ventri­cular wall and septum pellucidum'''
+
**[[Adult brain tumors classification|Central neurocytoma]]
***[[Ependymoma]]
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**[[Subependymal giant cell astrocytoma]]
***[[Adult brain tumors classification|Central neurocytoma]]
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*'''Neoplasms of the choroid plexus'''
***[[Subependymal giant cell astrocytoma]]
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**[[Choroid plexus]] papilloma and [[carcinoma]]
**'''Neoplasms of the choroid plexus'''
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*'''Others'''
***[[Choroid plexus papilloma and carcinoma]]
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**[[meningioma|Intraventricular meningioma]]
**'''Others'''
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**[[intracerebral metastasis|Intraventricular metastasis]]
***[[meningioma|Intraventricular meningioma]]
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**[[Oligodendroglioma]]
***[[intracerebral metastasis|Intraventricular metastasis]]
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**[[Pilocytic astrocytoma]]
***[[Oligodendroglioma]]
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**[[Glioblastoma multiforme]]
***[[Pilocytic astrocytoma]]
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**[[Medulloblastoma]]
***[[Glioblastoma multiforme]]
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**[[teratoma|Intraventricular teratoma]]
***[[Medulloblastoma]]
 
***[[teratoma|Intraventricular teratoma]]
 
  
 
==Epidemiology and Demographics==
 
==Epidemiology and Demographics==
===Prevalence===
+
===Frequency and Incidence===
*Subependymoma constitutes approximately 1% of all intracranial tumors.<ref name="pmid23382616">{{cite journal| author=Saad AF, Bidiwala SB, Layton KF, Snipes GJ, Opatowsky MJ| title=Fourth ventricular subependymoma presenting as worsening headache. | journal=Proc (Bayl Univ Med Cent) | year= 2013 | volume= 26 | issue= 1 | pages= 52-4 | pmid=23382616 | doi= | pmc=PMC3523772 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23382616 }} </ref>
+
*The frequency of [[asymptomatic]] subependymomas was 0.4% in 1,000 serial routine necropsies and 0.7% in [[symptomatic]] subependymomas from 1,000 serial [[Surgery|surgical]] specimens of [[Cranium|intracranial]] [[Neoplasm|neoplasms]].<ref name="pmid2929389">{{cite journal| author=Matsumura A, Ahyai A, Hori A, Schaake T| title=Intracerebral subependymomas. Clinical and neuropathological analyses with special reference to the possible existence of a less benign variant. | journal=Acta Neurochir (Wien) | year= 1989 | volume= 96 | issue= 1-2 | pages= 15-25 | pmid=2929389 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2929389  }} </ref>
 +
* The [[incidence]] of subependymoma was estimated to be 0.7 cases per 100,000 individuals with [[Pathological|pathologically]] proven [[Cranium|intracranial]] [[Neoplasm|neoplasms]].<ref name="pmid22121350">{{cite journal| author=Kurukumbi M, Muley A, Ramidi G, Wynn Z, Trouth AJ| title=A rare case of subependymoma with an atypical presentation: a case report. | journal=Case Rep Neurol | year= 2011 | volume= 3 | issue= 3 | pages= 227-32 | pmid=22121350 | doi=10.1159/000333061 | pmc=3223030 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22121350 }} </ref>
 +
===Age===
 +
 
 +
*[[Patient|Patients]] of all age groups may develop subependymoma.
  
===Age===
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*Subependymoma is a rare [[disease]] that tends to affect mi<nowiki/>ddle-aged adults and the elderly population (typically in the 5th to 6th decades).<ref name="epidemiosubepe1">Epidemiology of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref>
*Subependymoma is a rare disease that tends to affect middle-aged adults and the elderly population (typically 5th to 6th decades).<ref name="epidemiosubepe1">Epidemiology of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref>
 
  
 
===Gender===
 
===Gender===
*Males are more commonly affected with subependymoma than females. The male to female ratio is approximately 2.3 to 1.<ref name="epidemiosubepe1">Epidemiology of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref>
+
*Males are more commonly affected with subependymoma than females.  
 +
*The male to female ratio is approximately 2.3 to 1.<ref name="epidemiosubepe1" />
  
==Natural History, Complications and Prognosis==
+
==Risk Factors==
===Natural History===
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The [[Risk factor|risk factors]] in the development of subependymoma are not well defined.
*If left untreated, patients with subependymoma may progress to develop [[seizures]] and [[obstructive hydrocephalus]].<ref name="clinicalpresentationsubep1">Clinical presentation of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref>
 
*Subependymoma is a slow growing tumor with an indolent course.
 
  
===Complications===
+
== Natural History, Complications, and Prognosis ==
*[[Obstructive hydrocephalus]] is a common complication of subependymoma.<ref name="clinicalpresentationsubep1">Clinical presentation of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref>
 
  
===Prognosis===
+
*If left untreated, [[patients]] with subependymoma may progress to develop [[seizures]] and [[obstructive hydrocephalus]].<ref name="clinicalpresentationsubep1">Clinical presentation of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref>
*The prognosis of subependymoma is excellent with complete excision of the [[tumor]].<ref name="pmid23382616">{{cite journal| author=Saad AF, Bidiwala SB, Layton KF, Snipes GJ, Opatowsky MJ| title=Fourth ventricular subependymoma presenting as worsening headache. | journal=Proc (Bayl Univ Med Cent) | year= 2013 | volume= 26 | issue= 1 | pages= 52-4 | pmid=23382616 | doi= | pmc=PMC3523772 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23382616 }} </ref>
+
*Subependymoma is a slow-growing [[tumor]] with an indolent course.<ref name="pmid16639322">{{cite journal| author=Ragel BT, Osborn AG, Whang K, Townsend JJ, Jensen RL, Couldwell WT| title=Subependymomas: an analysis of clinical and imaging features. | journal=Neurosurgery | year= 2006 | volume= 58 | issue= 5 | pages= 881-90; discussion 881-90 | pmid=16639322 | doi=10.1227/01.NEU.0000209928.04532.09 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16639322  }} </ref><ref name="pmid28804038">{{cite journal| author=D'Amico RS, Praver M, Zanazzi GJ, Englander ZK, Sims JS, Samanamud JL et al.| title=Subependymomas Are Low-Grade Heterogeneous Glial Neoplasms Defined by Subventricular Zone Lineage Markers. | journal=World Neurosurg | year= 2017 | volume= 107 | issue= | pages= 451-463 | pmid=28804038 | doi=10.1016/j.wneu.2017.08.009 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28804038 }} </ref>
  
==History and Symptoms==
+
*[[Obstructive hydrocephalus]] is a common complication of subependymoma.<ref name="clinicalpresentationsubep1" />
===History===
 
*When evaluating a patient for subependymoma, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history review.
 
  
 +
*The [[prognosis]] of subependymoma is excellent with complete [[excision]] of the [[tumor]].<ref name="pmid23382616" /><ref name="pmid10320142">{{cite journal| author=Prayson RA, Suh JH| title=Subependymomas: clinicopathologic study of 14 tumors, including comparative MIB-1 immunohistochemical analysis with other ependymal neoplasms. | journal=Arch Pathol Lab Med | year= 1999 | volume= 123 | issue= 4 | pages= 306-9 | pmid=10320142 | doi=10.1043/0003-9985(1999)123<0306:S>2.0.CO;2 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10320142  }} </ref>
 +
*Common [[Complication (medicine)|complications]] of subependymoma are [[hydrocephalus]] and focal [[Neurology|neurological]] deficits due to [[Mass effect (medicine)|mass effect]].<ref name="pmid22747714">{{cite journal| author=Jain A, Amin AG, Jain P, Burger P, Jallo GI, Lim M et al.| title=Subependymoma: clinical features and surgical outcomes. | journal=Neurol Res | year= 2012 | volume= 34 | issue= 7 | pages= 677-84 | pmid=22747714 | doi=10.1179/1743132812Y.0000000064 | pmc=4618470 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22747714  }} </ref>
 +
 +
== Diagnosis ==
 
===Symptoms===
 
===Symptoms===
*Typically patients of subependymoma are asymptomatic and small lesions are discovered incidentally.
+
*Typically [[Patient|patients]] of subependymoma are [[asymptomatic]] and small [[Lesion|lesions]] are discovered incidentally.
*Symptoms of subependymoma include:<ref name="symptsubependymoma1">{{cite journal|last= KE|first= Changshu|title= Subependymoma: a case report and the review of literatures |doi=10.3969/j.issn.1672-6731.2011.01.021|url= http://www.cjcnn.org/index.php/cjcnn/article/view/323}}</ref>
+
*[[Symptoms]] of subependymoma include:<ref name="symptsubependymoma1">{{cite journal|last= KE|first= Changshu|title= Subependymoma: a case report and the review of literatures |doi=10.3969/j.issn.1672-6731.2011.01.021|url= http://www.cjcnn.org/index.php/cjcnn/article/view/323}}</ref><ref name="pmid2278665">{{cite journal| author=Park YK, Choi WS, Leem W, Kim YW, Yang MH| title=Symptomatic subependymoma--a case report. | journal=J Korean Med Sci | year= 1990 | volume= 5 | issue= 2 | pages= 111-5 | pmid=2278665 | doi=10.3346/jkms.1990.5.2.111 | pmc=3053733 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2278665  }} </ref><ref name="pmid22747714">{{cite journal| author=Jain A, Amin AG, Jain P, Burger P, Jallo GI, Lim M et al.| title=Subependymoma: clinical features and surgical outcomes. | journal=Neurol Res | year= 2012 | volume= 34 | issue= 7 | pages= 677-84 | pmid=22747714 | doi=10.1179/1743132812Y.0000000064 | pmc=4618470 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22747714  }} </ref>
 
**'''Symptoms due to elevated intracranial pressure'''
 
**'''Symptoms due to elevated intracranial pressure'''
 
***[[Headache]]
 
***[[Headache]]
Line 74: Line 99:
 
***[[memory loss|Transient loss of memory]]
 
***[[memory loss|Transient loss of memory]]
  
==CT==
+
:*
*Head CT scan is helpful in the diagnosis of subependymoma. On CT scan, subependymoma is characterized by:<ref name="radiographicfeaturessubepenedymoma1">Radiographic features of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref>
+
:*Iso- and hypodense intraventricular mass
+
=== Physical Examination ===
:*Positive mass effect
+
 
:*No enhancement
+
*[[Patient|Patients]] with subependymoma usually appear normal.
:*If large, it may have cystic or even calcific components
+
*[[Physical examination]] may be remarkable for:<ref name="pmid23607015">{{cite journal| author=Bokhari R, Ghanem A, Alahwal M, Baeesa S| title=Primary isolated lymphoma of the fourth ventricle in an immunocompetent patient. | journal=Case Rep Oncol Med | year= 2013 | volume= 2013 | issue=  | pages= 614658 | pmid=23607015 | doi=10.1155/2013/614658 | pmc=3625557 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23607015  }} </ref><ref name="pmid17761056">{{cite journal| author=Hamilton W, Kernick D| title=Clinical features of primary brain tumours: a case-control study using electronic primary care records. | journal=Br J Gen Pract | year= 2007 | volume= 57 | issue= 542 | pages= 695-9 | pmid=17761056 | doi= | pmc=2151783 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17761056  }} </ref><ref name="pmid16547083">{{cite journal| author=Wilne SH, Ferris RC, Nathwani A, Kennedy CR| title=The presenting features of brain tumours: a review of 200 cases. | journal=Arch Dis Child | year= 2006 | volume= 91 | issue= 6 | pages= 502-6 | pmid=16547083 | doi=10.1136/adc.2005.090266 | pmc=2082784 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16547083  }} </ref><ref name="pmid26926614">{{cite journal| author=Perkins A, Liu G| title=Primary Brain Tumors in Adults: Diagnosis and Treatment. | journal=Am Fam Physician | year= 2016 | volume= 93 | issue= 3 | pages= 211-7 | pmid=26926614 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26926614  }} </ref><ref name="pmid22747714">{{cite journal| author=Jain A, Amin AG, Jain P, Burger P, Jallo GI, Lim M et al.| title=Subependymoma: clinical features and surgical outcomes. | journal=Neurol Res | year= 2012 | volume= 34 | issue= 7 | pages= 677-84 | pmid=22747714 | doi=10.1179/1743132812Y.0000000064 | pmc=4618470 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22747714  }} </ref><ref name="pmid25361493">{{cite journal| author=Bi Z, Ren X, Zhang J, Jia W| title=Clinical, radiological, and pathological features in 43 cases of intracranial subependymoma. | journal=J Neurosurg | year= 2015 | volume= 122 | issue= 1 | pages= 49-60 | pmid=25361493 | doi=10.3171/2014.9.JNS14155 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25361493  }} </ref>
:*No [[vasogenic edema]]
+
 
 +
:*Abnormal [[pupillary reflex]]
 +
:*[[Visual field]] defects
 +
:*[[Gait]] changes
 +
:*Bilateral [[Babinski sign]]
 +
:*Depressed [[Glasgow coma score|Glasgow coma score (GCS)]]
 +
:*Decreased [[muscle]] strength
 +
:*Decreased [[Deep tendon reflex|deep tendon reflexes]]
 +
:*[[Sensation]] defects
 +
:*[[Hearing (sense)|Hearing]] problems and abnormal [[Rinne test|Rinne]] and [[Weber test|Weber tests]]
 +
 
 +
=== Laboratory Findings ===
 +
There are no specific laboratory findings associated with subependymoma.
 +
 
 +
===Electrocardiogram===
 +
There are no [[The electrocardiogram|ECG]] findings associated with subependymoma.
 +
 
 +
=== X-ray ===
 +
There are no [[X-rays|x-ray]] findings associated with subependymoma.
 +
 
 +
===Echocardiography or Ultrasound===
 +
There are no [[echocardiography]]/[[ultrasound]] findings associated with sybependymoma.
 +
 
 +
===CT scan===
 +
[[Head]] [[Computed tomography|CT scan]] is helpful in the [[diagnosis]] of subependymoma. On [[Computed tomography|CT scan]], subependymoma is characterized by:<ref name="radiographicfeaturessubepenedymoma1">Radiographic features of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016</ref>
  
==MRI==
+
* Iso- and hypodense intraventricular mass
*Brain MRI is helpful in the diagnosis of subependymoma. On MRI, subependymoma is characterized by:
+
* Positive [[Mass effect (medicine)|mass effect]]
 +
* No enhancement
 +
* If large, it may have [[cystic]] or even [[Calcification|calcific]] components
 +
* No [[vasogenic edema]]
  
 +
=== MRI ===
 +
[[Brain]] [[MRI]] is helpful in the [[diagnosis]] of subependymoma. On [[MRI]], subependymoma is characterized by:
 
{| style="border: 0px; font-size: 90%; margin: 3px; width:1000px"
 
{| style="border: 0px; font-size: 90%; margin: 3px; width:1000px"
|valign=top|
+
| valign="top" |
 
|+
 
|+
! style="background: #4479BA; width: 300px;" | {{fontcolor|#FFF|MRI component}}
+
! style="background: #4479BA; width: 300px;" |{{fontcolor|#FFF|MRI component}}
! style="background: #4479BA; width: 800px;" | {{fontcolor|#FFF|Findings}}
+
! style="background: #4479BA; width: 800px;" |{{fontcolor|#FFF|Findings}}
 
|-
 
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center |
+
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align="center" |
 
T1 weighted image
 
T1 weighted image
 
| style="padding: 5px 5px; background: #F5F5F5;" |
 
| style="padding: 5px 5px; background: #F5F5F5;" |
*Iso - hypointense compared to white matter
+
*Iso - hypointense compared to [[white matter]]
*Homogeneous but may be heterogeneous in larger lesions
+
*[[Homogeneous]] but may be [[heterogeneous]] in larger [[Lesion|lesions]]
 
|-
 
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
+
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align="center" |
 
T2 weighted image
 
T2 weighted image
 
| style="padding: 5px 5px; background: #F5F5F5;" |
 
| style="padding: 5px 5px; background: #F5F5F5;" |
*Hyperintense compared to adjacent white and grey matter
+
*Hyperintense compared to adjacent [[White matter|white]] and [[grey matter]]
*Heterogeneity may be seen in larger lesions, with susceptibility related signal drop out due to calcifications
+
*[[Heterogeneous|Heterogeneity]] may be seen in larger [[Lesion|lesions]], with susceptibility related signal drop-out due to [[Calcification|calcifications]]
*No adjacent parenchymal edema (as no brain invasion is present)
+
*No adjacent [[Parenchyma|parenchymal]] [[edema]] (as no [[brain]] [[Invasive (medical)|invasion]] is present)
 
|-
 
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
+
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align="center" |
 
T1 weighted image with contrast
 
T1 weighted image with contrast
 
| style="padding: 5px 5px; background: #F5F5F5;" |
 
| style="padding: 5px 5px; background: #F5F5F5;" |
Line 110: Line 164:
 
|}
 
|}
  
==Biopsy==
+
=== Other Imaging Findings ===
*[[Biopsy]] of the subependymoma tumor, taken through a needle during a simple surgical procedure, helps to confirm the diagnosis.<ref name="diagnosisse1">Diagnosis of subependymoma. Wikipedia 2016. https://en.wikipedia.org/wiki/Subependymoma. Accessed on January 8, 2016</ref>
+
There are no other [[imaging]] findings associated with subependymoma.
 +
 
 +
=== Other Diagnostic Studies ===
 +
There are no other [[Diagnosis|diagnostic]] studies associated with subependymoma.
 +
 
 +
=== Medical Therapy ===
 +
There is no medical therapy available for the treatment of subependymoma.
 +
 
 +
=== Surgery ===
 +
[[Surgery]] is the mainstay of [[therapy]] for subependymoma. Incidental intraventricular subependymoma can be managed conservatively through MRI surveillance. [[Resection|Surgical resection]] is indicated for:<ref name="pmid22747714" /><ref name="pmid28232153">{{cite journal| author=Nguyen HS, Doan N, Gelsomino M, Shabani S| title=Intracranial Subependymoma: A SEER Analysis 2004-2013. | journal=World Neurosurg | year= 2017 | volume= 101 | issue=  | pages= 599-605 | pmid=28232153 | doi=10.1016/j.wneu.2017.02.019 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28232153  }} </ref><ref name="pmid29915887">{{cite journal| author=Varma A, Giraldi D, Mills S, Brodbelt AR, Jenkinson MD| title=Surgical management and long-term outcome of intracranial subependymoma. | journal=Acta Neurochir (Wien) | year= 2018 | volume= 160 | issue= 9 | pages= 1793-1799 | pmid=29915887 | doi=10.1007/s00701-018-3570-4 | pmc=6105212 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=29915887  }} </ref>
 +
 
 +
*[[Symptomatic]] [[Tumor|tumors]]
 +
*[[Tumors]] without a clear [[imaging]] [[diagnosis]]
 +
 
 +
=== Primary Prevention ===
 +
There are no established measures for the [[Prevention (medical)|secondary prevention]] of subependymoma.
 +
 
 +
=== Secondary Prevention ===
 +
There are no established measures for the [[Prevention (medical)|primary prevention]] of subependymoma.
  
==Treatment==
+
==References==
*The predominant therapy for subependymoma is [[surgery|surgical resection]].
+
{{Reflist|2}}
 +
 +
[[Category:Pick One of 28 Approved]]
  
 +
__NOTOC__
 
==References==
 
==References==
 
{{reflist|2}}
 
{{reflist|2}}

Revision as of 04:31, 6 May 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Syed Musadiq Ali M.B.B.S.[2]Mohamadmostafa Jahansouz M.D.[3]Sujit Routray, M.D. [4]

Historical Perspective

Subependymoma was first discovered by Ilya Mark Scheinker, a Russian physician, in 1945.[1]

Classification

There is no established system for the classification of subependymoma.

Pathophysiology

Pathogenesis

Subependymoma arises from subependymal glial cells, although it can also arise from astrocytes from the subependymal plate, ependymal cells, and mixed ependymal and astrocytic cells.[2][3]

Gross Pathology

Subependymoma is most commonly seen in the fourth ventricle, but it can arise anywhere where there is ependyma. The distribution in the ventricular system is as follows:[4][3]

  • On gross pathology, subependymoma is characterized by a small, white to grey, firm, well circumscribed, solid, avascular mass attached to the ventricular wall by a narrow pedicle.[2][3]

Microscopic Pathology

On microscopic histopathological analysis, subependymoma is characterized by the following features:[4]

Immunohistochemistry

Subependymoma is characterized by positive tumor marker GFAP. Mixed populations of cells may be variably positive for:[4][5]

Causes

The cause of the development of subependymoma has not been identified.

Differentiating Subependymoma from Other Diseases

Subependymoma must be differentiated from:[6][3]

Epidemiology and Demographics

Frequency and Incidence

Age

  • Patients of all age groups may develop subependymoma.
  • Subependymoma is a rare disease that tends to affect middle-aged adults and the elderly population (typically in the 5th to 6th decades).[9]

Gender

  • Males are more commonly affected with subependymoma than females.
  • The male to female ratio is approximately 2.3 to 1.[9]

Risk Factors

The risk factors in the development of subependymoma are not well defined.

Natural History, Complications, and Prognosis

Diagnosis

Symptoms

Physical Examination

Laboratory Findings

There are no specific laboratory findings associated with subependymoma.

Electrocardiogram

There are no ECG findings associated with subependymoma.

X-ray

There are no x-ray findings associated with subependymoma.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with sybependymoma.

CT scan

Head CT scan is helpful in the diagnosis of subependymoma. On CT scan, subependymoma is characterized by:[20]

MRI

Brain MRI is helpful in the diagnosis of subependymoma. On MRI, subependymoma is characterized by:

MRI component Findings

T1 weighted image

T2 weighted image

T1 weighted image with contrast

  • Little or no enhancement

Other Imaging Findings

There are no other imaging findings associated with subependymoma.

Other Diagnostic Studies

There are no other diagnostic studies associated with subependymoma.

Medical Therapy

There is no medical therapy available for the treatment of subependymoma.

Surgery

Surgery is the mainstay of therapy for subependymoma. Incidental intraventricular subependymoma can be managed conservatively through MRI surveillance. Surgical resection is indicated for:[3][21][22]

Primary Prevention

There are no established measures for the secondary prevention of subependymoma.

Secondary Prevention

There are no established measures for the primary prevention of subependymoma.

References

  1. Kurian KM, Jones DT, Marsden F, Openshaw SW, Pearson DM, Ichimura K; et al. (2008). "Genome-wide analysis of subependymomas shows underlying chromosomal copy number changes involving chromosomes 6, 7, 8 and 14 in a proportion of cases". Brain Pathol. 18 (4): 469–73. doi:10.1111/j.1750-3639.2008.00148.x. PMC 2659379. PMID 18397339.
  2. 2.0 2.1 2.2 Saad AF, Bidiwala SB, Layton KF, Snipes GJ, Opatowsky MJ (2013). "Fourth ventricular subependymoma presenting as worsening headache". Proc (Bayl Univ Med Cent). 26 (1): 52–4. PMC 3523772. PMID 23382616.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Jain A, Amin AG, Jain P, Burger P, Jallo GI, Lim M; et al. (2012). "Subependymoma: clinical features and surgical outcomes". Neurol Res. 34 (7): 677–84. doi:10.1179/1743132812Y.0000000064. PMC 4618470. PMID 22747714.
  4. 4.0 4.1 4.2 Pathology of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016
  5. 5.0 5.1 D'Amico RS, Praver M, Zanazzi GJ, Englander ZK, Sims JS, Samanamud JL; et al. (2017). "Subependymomas Are Low-Grade Heterogeneous Glial Neoplasms Defined by Subventricular Zone Lineage Markers". World Neurosurg. 107: 451–463. doi:10.1016/j.wneu.2017.08.009. PMID 28804038.
  6. Intraventricular neoplasms and lesions. Dr Henry Knipe and Dr Vinod G Maller et al. Radiopaedia 2016. http://radiopaedia.org/articles/intraventricular-neoplasms-and-lesions. Accessed on January 12, 2016
  7. Matsumura A, Ahyai A, Hori A, Schaake T (1989). "Intracerebral subependymomas. Clinical and neuropathological analyses with special reference to the possible existence of a less benign variant". Acta Neurochir (Wien). 96 (1–2): 15–25. PMID 2929389.
  8. Kurukumbi M, Muley A, Ramidi G, Wynn Z, Trouth AJ (2011). "A rare case of subependymoma with an atypical presentation: a case report". Case Rep Neurol. 3 (3): 227–32. doi:10.1159/000333061. PMC 3223030. PMID 22121350.
  9. 9.0 9.1 Epidemiology of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016
  10. 10.0 10.1 Clinical presentation of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016
  11. Ragel BT, Osborn AG, Whang K, Townsend JJ, Jensen RL, Couldwell WT (2006). "Subependymomas: an analysis of clinical and imaging features". Neurosurgery. 58 (5): 881–90, discussion 881-90. doi:10.1227/01.NEU.0000209928.04532.09. PMID 16639322.
  12. Prayson RA, Suh JH (1999). "Subependymomas: clinicopathologic study of 14 tumors, including comparative MIB-1 immunohistochemical analysis with other ependymal neoplasms". Arch Pathol Lab Med. 123 (4): 306–9. doi:10.1043/0003-9985(1999)123<0306:S>2.0.CO;2. PMID 10320142.
  13. KE, Changshu. "Subependymoma: a case report and the review of literatures". doi:10.3969/j.issn.1672-6731.2011.01.021.
  14. Park YK, Choi WS, Leem W, Kim YW, Yang MH (1990). "Symptomatic subependymoma--a case report". J Korean Med Sci. 5 (2): 111–5. doi:10.3346/jkms.1990.5.2.111. PMC 3053733. PMID 2278665.
  15. Bokhari R, Ghanem A, Alahwal M, Baeesa S (2013). "Primary isolated lymphoma of the fourth ventricle in an immunocompetent patient". Case Rep Oncol Med. 2013: 614658. doi:10.1155/2013/614658. PMC 3625557. PMID 23607015.
  16. Hamilton W, Kernick D (2007). "Clinical features of primary brain tumours: a case-control study using electronic primary care records". Br J Gen Pract. 57 (542): 695–9. PMC 2151783. PMID 17761056.
  17. Wilne SH, Ferris RC, Nathwani A, Kennedy CR (2006). "The presenting features of brain tumours: a review of 200 cases". Arch Dis Child. 91 (6): 502–6. doi:10.1136/adc.2005.090266. PMC 2082784. PMID 16547083.
  18. Perkins A, Liu G (2016). "Primary Brain Tumors in Adults: Diagnosis and Treatment". Am Fam Physician. 93 (3): 211–7. PMID 26926614.
  19. Bi Z, Ren X, Zhang J, Jia W (2015). "Clinical, radiological, and pathological features in 43 cases of intracranial subependymoma". J Neurosurg. 122 (1): 49–60. doi:10.3171/2014.9.JNS14155. PMID 25361493.
  20. Radiographic features of subependymoma. Dr Bruno Di Muzio and A.Prof Frank Gaillard et al. Radiopaedia 2016. http://radiopaedia.org/articles/subependymoma. Accessed on January 12, 2016
  21. Nguyen HS, Doan N, Gelsomino M, Shabani S (2017). "Intracranial Subependymoma: A SEER Analysis 2004-2013". World Neurosurg. 101: 599–605. doi:10.1016/j.wneu.2017.02.019. PMID 28232153.
  22. Varma A, Giraldi D, Mills S, Brodbelt AR, Jenkinson MD (2018). "Surgical management and long-term outcome of intracranial subependymoma". Acta Neurochir (Wien). 160 (9): 1793–1799. doi:10.1007/s00701-018-3570-4. PMC 6105212. PMID 29915887.


References


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