Ependymoma overview: Difference between revisions

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Latest revision as of 18:31, 26 September 2019

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 the third most common neuroepithelial tumor of the central nervous system (CNS) in childhood. It arises for ependymal cells of the central nervous system and is dominated by intracranial mass. The World Health Organization (WHO) classification of CNS tumors defines several histopathological variants of ependymoma (grade I, II, III). 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). There are no established causes for ependymomas. Ependymoma must be differentiated from medulloblastoma, choroid plexus papilloma, and glioblastoma. 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.Symptoms of ependymoma include headache, nausea, vomiting, blurry or double vision, drowsiness (after several hours of the above symptoms), irritability, ataxia, neck pain, cranial nerve palsies, seizures, focal neurologic deficits, back pain, lower extremity weakness, bowel and bladder dysfunction. 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. The predominant therapy for ependymoma is surgical resection. Adjunctive chemoradiation may be required.

Classification

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

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).

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.^[1]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.

Risk Factors

Common risk factors in the development of ependymoma are children with certain hereditary diseases (neurofibromatosis type II and Turcot syndrome), 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.

Other Diagnostic Studies

Other diagnostic studies for ependymoma include EEG, which demonstrates various abnormalities, and cerebrospinal fluid analysis, which demonstrates positive cytology.

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.

References

↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq

Template:WikiDoc Sources

[cancergov-1] National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq

[1]

@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Ependymoma}}
-{{CMG}}
+{{CMG}} {{AE}} {{AAM}}
 ==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 [[spine|spinal]]. The common location of intracranial ependymoma is the [[fourth ventricle]].  Rarely, ependymoma can occur in the [[pelvic cavity]].
+'''Ependymoma''' is the third most common neuroepithelial tumor of the [[central nervous system]] (CNS) in childhood. It arises for ependymal cells of the central nervous system and is dominated by [[intracranial mass]]. The World Health Organization (WHO) classification of CNS tumors defines several [[histopathological]] variants of ependymoma (grade I, II, III). 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]]''). There are no established causes for ependymomas. Ependymoma must be differentiated from [[medulloblastoma]], [[choroid plexus papilloma]], and [[glioblastoma]]. 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]].Symptoms of ependymoma include [[headache]], [[nausea]], [[vomiting]], blurry or [[double vision]], [[drowsiness]] (after several hours of the above symptoms), [[irritability]], [[ataxia]], neck pain, [[cranial nerve palsies]], [[seizures]], [[focal neurologic deficits]], [[back pain]], lower extremity weakness, bowel and bladder dysfunction. 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]]. The predominant therapy for ependymoma is surgical resection. Adjunctive chemoradiation may be required.
-[[Syringomyelia]] can be caused by an ependymona.
+==Classification==
-Ependymomas are also seen with Neurofibromatosis Type II.
+Ependymoma may be classified into several subtypes based on [[WHO]] classification (grade I, II, III) and the site of origin.
+==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]]'').
+==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.
+==Risk Factors==
+Common risk factors in the development of ependymoma are children with certain hereditary diseases ([[neurofibromatosis]] type II and Turcot syndrome), 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.
+===Other Diagnostic Studies===
+Other diagnostic studies for ependymoma include [[EEG]], which demonstrates various abnormalities, and [[cerebrospinal fluid]] analysis, which demonstrates positive [[cytology]].
+==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]].
 ==References==
 {{reflist|2}}
 [[Category:Disease]]
 [[Category:Types of cancer]]
 [[Category:Rare diseases]]
-[[Category:Oncology]]
 [[Category:Neurology]]
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