Ependymoma overview: Difference between revisions
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{{Ependymoma}} | {{Ependymoma}} | ||
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==Overview== | ==Overview== | ||
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[[Syringomyelia]] can be caused by an ependymona. | [[Syringomyelia]] can be caused by an ependymona. | ||
Ependymomas are also seen with Neurofibromatosis Type II. | Ependymomas are also seen with Neurofibromatosis Type II. | ||
==Classification== | |||
Ependymoma may be classified into several subtypes based on [[WHO]] classification (Grade I, II, III) and the site of origin.<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> | |||
==Pathology== | |||
On [[gross pathology]], a well-encapsulated [[tumor]] arises from the floor of the [[fourth ventricle]], situated in the lower back portion of the [[brain]] is a characteristic finding of ependymoma. On [[microscopic]] histopathological analysis, perivascular pseudorosettes are characteristic findings of ependymoma. Development of ependymoma is the result of multiple genetic mutations (''ERBB2'', ''ERBB4'', ''[[MMP2]]'', ''MMP14'', ''[[NOTCH1]]'', and ''[[MEN1]]'').<ref name=Epe>Kumar, et al. (2005). The Central Nervous System. Pathologic Basis of Disease. 7th Edition. Philadelphia: Elsevier Saunders.</ref><ref name=Wikipedia> ependymoma https://en.wikipedia.org/wiki/Pediatric_ependymoma#Cell_of_origin URL Accessed on 10/08/2015</ref> | |||
==Causes== | |||
There are no established causes for ependymomas. | |||
==Epidemiology and Demographics== | |||
The incidence of ependymoma is approximately 0.05 to 0.08 per 100,000 individuals in the United States.<ref name="cancergov">National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq</ref>The posterior fossa tumours tend to present more commonly in the paediatric age group (mean age at diagnosis is 6 years of age)Men and women are affected equally by ependymomal tumors<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> | |||
==Risk Factors== | |||
Common risk factors in the development of ependymoma are children with certain hereditary diseases ([[neurofibromatosis]] type II and Turcot syndrome), ''ERBB2'', ''ERBB4'', and human telomerase reverse transcriptase ''TERT'' gene expression, over-expression of [[kinetochore]] proteins, and down-regulation of [[metallothionein|metallothioneins]]. | |||
==Differentiating ependymoma from other diseases== | |||
Ependymoma must be differentiated from [[medulloblastoma]], [[choroid plexus papilloma]], and [[glioblastoma]]. | |||
==Natural History, Complication and Prognosis== | |||
If left untreated, patients with ependymoma may progress to develop [[nausea]], [[vomiting]], [[headache]], and [[irritability]]. Common complications of ependymoma include [[seizure]], [[hydrocephalus]], [[paralysis|muscle paralysis]], and [[speech problems]]. | |||
==Diagnosis== | |||
===History and Symptoms=== | |||
Symptoms of ependymoma include [[headache]], [[nausea]], and [[irritability]]. | |||
===Physical Examination=== | |||
Patients with ependymoma usually appear well. Physical examination of patients with ependymoma is usually remarkable for [[altered mental status]], [[spasticity]], and [[muscle weakness]]. | |||
===Staging=== | |||
There is no established system for the staging of ependymoma. | |||
===Laboratory Findings=== | |||
There are no diagnostic lab findings associated with ependymoma. | |||
===CT=== | |||
Head CT scan may be diagnostic of ependymoma. Findings on CT scan suggestive of ependymoma include [[heterogeneous|heterogeneous mass]] with [[calcification|coarse calcification]], [[solid|solid component]], and [[cystic|cystic component]]. | |||
===MRI=== | |||
Brain MRI may be diagnostic of ependymoma. Finding on brain MRI suggestive of ependymoma include large mixed cystic/solid lesion with [[haemorrhage]] and [[fluid]] which may indicate areas of [[necrosis]]. | |||
===Ultrasound=== | |||
Intraoperative ultrasound is used in intradural spinal ependymomas.<ref name="pmid21507563">{{cite journal| author=Zhou H, Miller D, Schulte DM, Benes L, Bozinov O, Sure U et al.| title=Intraoperative ultrasound assistance in treatment of intradural spinal tumours. | journal=Clin Neurol Neurosurg | year= 2011 | volume= 113 | issue= 7 | pages= 531-7 | pmid=21507563 | doi=10.1016/j.clineuro.2011.03.006 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21507563 }} </ref> | |||
===Other Diagnostic Studies=== | |||
Other diagnostic studies for ependymoma include [[EEG]], which demonstrates various abnormalities, and [[cerebrospinal fluid]] analysis, which demonstrates positive [[cytology]].<ref name="pmid20463607">{{cite journal| author=Moreno L, Pollack IF, Duffner PK, Geyer JR, Grill J, Massimino M et al.| title=Utility of cerebrospinal fluid cytology in newly diagnosed childhood ependymoma. | journal=J Pediatr Hematol Oncol | year= 2010 | volume= 32 | issue= 6 | pages= 515-8 | pmid=20463607 | doi=10.1097/MPH.0b013e3181d7adf5 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20463607 }} </ref> | |||
==Treatment== | |||
===Medical Therapy=== | |||
The predominant therapy for ependymoma is surgical resection. Adjunctive chemoradiation may be required. | |||
===Surgery=== | |||
Surgery is the main stay of treatment for myxopapillary ependymoma ([[WHO]] grade 1), subependymoma ([[WHO]] grade 1), ependymoma ([[WHO]] grade I), and anaplastic ependymoma ([[WHO]] grade III). | |||
===Primary Prevention=== | |||
There are no primary preventive measures available for ependymoma. | |||
===Secondary Prevention=== | |||
Secondary prevention strategies following ependymoma include regular clinical assessment and [[neuroimaging]].<ref name=Cancergov> Ependymoma. http://www.cancer.gov/types/brain/hp/child-ependymoma-treatment-pdq#link/_291_toc. URL Accessed on 10 07, 2015</ref> | |||
==References== | ==References== | ||
{{reflist|2}} | {{reflist|2}} | ||
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[[Category:Types of cancer]] | [[Category:Types of cancer]] | ||
[[Category:Rare diseases]] | [[Category:Rare diseases]] | ||
[[Category:Neurology]] | [[Category:Neurology]] | ||
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Revision as of 17:30, 13 October 2015
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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 is a tumor that arises from the ependyma, a tissue of the central nervous system. Usually, in children 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 ependymona. Ependymomas are also seen with Neurofibromatosis Type II.
Classification
Ependymoma may be classified into several subtypes based on WHO classification (Grade I, II, III) and the site of origin.[1]
Pathology
On gross pathology, a well-encapsulated tumor arises from the floor of the fourth ventricle, situated in the lower back portion of the brain is a characteristic finding of ependymoma. On microscopic histopathological analysis, perivascular pseudorosettes are characteristic findings of ependymoma. Development of ependymoma is the result of multiple genetic mutations (ERBB2, ERBB4, MMP2, MMP14, NOTCH1, and MEN1).[2][3]
Causes
There are no established causes for ependymomas.
Epidemiology and Demographics
The incidence of ependymoma is approximately 0.05 to 0.08 per 100,000 individuals in the United States.[4]The posterior fossa tumours tend to present more commonly in the paediatric age group (mean age at diagnosis is 6 years of age)Men and women are affected equally by ependymomal tumors[1]
Risk Factors
Common risk factors in the development of ependymoma are children with certain hereditary diseases (neurofibromatosis type II and Turcot syndrome), ERBB2, ERBB4, and human telomerase reverse transcriptase TERT gene expression, over-expression of kinetochore proteins, and down-regulation of metallothioneins.
Differentiating ependymoma from other diseases
Ependymoma must be differentiated from medulloblastoma, choroid plexus papilloma, and glioblastoma.
Natural History, Complication and Prognosis
If left untreated, patients with ependymoma may progress to develop nausea, vomiting, headache, and irritability. Common complications of ependymoma include seizure, hydrocephalus, muscle paralysis, and speech problems.
Diagnosis
History and Symptoms
Symptoms of ependymoma include headache, nausea, and irritability.
Physical Examination
Patients with ependymoma usually appear well. Physical examination of patients with ependymoma is usually remarkable for altered mental status, spasticity, and muscle weakness.
Staging
There is no established system for the staging of ependymoma.
Laboratory Findings
There are no diagnostic lab findings associated with ependymoma.
CT
Head CT scan may be diagnostic of ependymoma. Findings on CT scan suggestive of ependymoma include heterogeneous mass with coarse calcification, solid component, and cystic component.
MRI
Brain MRI may be diagnostic of ependymoma. Finding on brain MRI suggestive of ependymoma include large mixed cystic/solid lesion with haemorrhage and fluid which may indicate areas of necrosis.
Ultrasound
Intraoperative ultrasound is used in intradural spinal ependymomas.[5]
Other Diagnostic Studies
Other diagnostic studies for ependymoma include EEG, which demonstrates various abnormalities, and cerebrospinal fluid analysis, which demonstrates positive cytology.[6]
Treatment
Medical Therapy
The predominant therapy for ependymoma is surgical resection. Adjunctive chemoradiation may be required.
Surgery
Surgery is the main stay of treatment for myxopapillary ependymoma (WHO grade 1), subependymoma (WHO grade 1), ependymoma (WHO grade I), and anaplastic ependymoma (WHO grade III).
Primary Prevention
There are no primary preventive measures available for ependymoma.
Secondary Prevention
Secondary prevention strategies following ependymoma include regular clinical assessment and neuroimaging.[7]
References
- ↑ 1.0 1.1 .Ependymomas Dr Bruno Di Muzio and Dr Frank Gaillard Gold Supporter since June 24, 2015">. Radiopaedia.org 2015.http://radiopaedia.org/articles/ependymoma
- ↑ Kumar, et al. (2005). The Central Nervous System. Pathologic Basis of Disease. 7th Edition. Philadelphia: Elsevier Saunders.
- ↑ ependymoma https://en.wikipedia.org/wiki/Pediatric_ependymoma#Cell_of_origin URL Accessed on 10/08/2015
- ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq
- ↑ Zhou H, Miller D, Schulte DM, Benes L, Bozinov O, Sure U; et al. (2011). "Intraoperative ultrasound assistance in treatment of intradural spinal tumours". Clin Neurol Neurosurg. 113 (7): 531–7. doi:10.1016/j.clineuro.2011.03.006. PMID 21507563.
- ↑ Moreno L, Pollack IF, Duffner PK, Geyer JR, Grill J, Massimino M; et al. (2010). "Utility of cerebrospinal fluid cytology in newly diagnosed childhood ependymoma". J Pediatr Hematol Oncol. 32 (6): 515–8. doi:10.1097/MPH.0b013e3181d7adf5. PMID 20463607.
- ↑ Ependymoma. http://www.cancer.gov/types/brain/hp/child-ependymoma-treatment-pdq#link/_291_toc. URL Accessed on 10 07, 2015