Gemistocytic astrocytoma: Difference between revisions

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==Overview==
==Overview==
Gemistocytic astrocytoma is a histologic subtype of [[low grade astrocytoma]], with a poorer prognosis than other matched WHO grade II astrocytic tumors. Gemistocytic astrocytomas are characterized by a significant gemistocytes population, which are large cells with their cytoplasm filled with eosinophilic material displacing the nucleus eccentrically.
Gemistocytic astrocytoma is a [[Histology|histologic]] subtype of [[low grade astrocytoma]], with a poorer [[prognosis]] than other matched [[World Health Organization|WHO]] [[Grading (tumors)|grade]] II [[Astrocyte|astrocytic]] [[Tumor|tumors]]. Gemistocytic astrocytoma is characterized by a significant [[gemistocytes]] population, which are large astrocytes with their [[cytoplasm]] filled with [[eosinophilic]] material displacing the [[Cell nucleus|nucleus]] eccentrically.


==Historical Perspective==
==Historical Perspective==
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== Classification ==
== Classification ==
Gemistocytic astrocytoma is a subtype of astrocytoma and is included in the classification of astrocytoma. For more information about the classification of astrocytoma, [[Astrocytoma classification|click here]].
Gemistocytic astrocytoma is a subtype of [[astrocytoma]] and is included in the [[classification]] of [[astrocytoma]]. For more information about the [[classification]] of [[astrocytoma]], [[Astrocytoma classification|click here]].


==Pathophysiology==
==Pathophysiology==


*Gemistocytic astrocytoma is characterized by a significant gemistocyte population, which are large cells with their cytoplasm filled with eosinophilic material displacing the nucleus eccentrically.
*Gemistocytic astrocytoma is characterized by a significant [[Gemistocytes|gemistocyte]] population, which are large [[Astrocyte|astrocytes]] with their [[cytoplasm]] filled with [[eosinophilic]] material displacing the [[Cell nucleus|nucleus]] eccentrically.
*It is important to note that other gliomas can have occasional gemistocytes, without being designated a gemistocytic astrocytoma. A cut off of 20% of the tumor cells being gemistocytes may be used before designating it as a gemistocytic astrocytoma.
*It is important to note that other [[Glioma|gliomas]] can have occasional [[gemistocytes]], without being designated a gemistocytic astrocytoma. A cut off of 20% of the [[Tumor cell|tumor cells]] being [[gemistocytes]] is used before designating it as a gemistocytic astrocytoma.
*Other CNS tumors and conditions that have gemistocytes or gemistocyte-like cells include:<ref name="TihanVohra2005">{{cite journal|last1=Tihan|first1=Tarik|last2=Vohra|first2=Poonam|last3=Berger|first3=Mitchel S.|last4=Keles|first4=G. Evren|title=Definition and Diagnostic Implications of Gemistocytic Astrocytomas: A Pathological Perspective|journal=Journal of Neuro-Oncology|volume=76|issue=2|year=2005|pages=175–183|issn=0167-594X|doi=10.1007/s11060-005-4897-2}}</ref><ref>{{cite book | last = Prayson | first = Richard | title = Brain tumors | publisher = Demos Medical Pub | location = New York | year = 2010 | isbn = 1933864699 }}</ref>
*Other [[CNS tumor|CNS tumors]] and conditions that have [[gemistocytes]] or [[Gemistocytes|gemistocyte]]-like [[Cell (biology)|cells]] include:<ref name="TihanVohra2005">{{cite journal|last1=Tihan|first1=Tarik|last2=Vohra|first2=Poonam|last3=Berger|first3=Mitchel S.|last4=Keles|first4=G. Evren|title=Definition and Diagnostic Implications of Gemistocytic Astrocytomas: A Pathological Perspective|journal=Journal of Neuro-Oncology|volume=76|issue=2|year=2005|pages=175–183|issn=0167-594X|doi=10.1007/s11060-005-4897-2}}</ref><ref>{{cite book | last = Prayson | first = Richard | title = Brain tumors | publisher = Demos Medical Pub | location = New York | year = 2010 | isbn = 1933864699 }}</ref>


:*[[Oligodendroglioma]] with "minigemistocytes"
:*[[Oligodendroglioma]] with "minigemistocytes"
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:*[[Subependymal giant cell astrocytoma]]
:*[[Subependymal giant cell astrocytoma]]
:*Astroblastoma
:*Astroblastoma
:*Reactive [[gliosis]] in inflammation, infection, or demyelinating disease
:*Reactive [[gliosis]] in [[inflammation]], [[infection]], or [[demyelinating disease]]
:*Toxic/metabolic leukoencephalopathy
:*[[Toxic encephalopathy|Toxic/metabolic leukoencephalopathy]]
 
*On [[gross pathology]], gemistocytic astrocytoma is characterized by:<ref name="TihanVohra2005">{{cite journal|last1=Tihan|first1=Tarik|last2=Vohra|first2=Poonam|last3=Berger|first3=Mitchel S.|last4=Keles|first4=G. Evren|title=Definition and Diagnostic Implications of Gemistocytic Astrocytomas: A Pathological Perspective|journal=Journal of Neuro-Oncology|volume=76|issue=2|year=2005|pages=175–183|issn=0167-594X|doi=10.1007/s11060-005-4897-2}}</ref>


*On gross pathology, gemistocytic astrocytoma is characterized by:<ref name="TihanVohra2005">{{cite journal|last1=Tihan|first1=Tarik|last2=Vohra|first2=Poonam|last3=Berger|first3=Mitchel S.|last4=Keles|first4=G. Evren|title=Definition and Diagnostic Implications of Gemistocytic Astrocytomas: A Pathological Perspective|journal=Journal of Neuro-Oncology|volume=76|issue=2|year=2005|pages=175–183|issn=0167-594X|doi=10.1007/s11060-005-4897-2}}</ref>
:*Gray-tan mass
:*Gray-tan mass
:*Well-defined borders
:*Well-defined borders
:*Soft texture
:*Soft texture
:*Cystic architecture
:*[[Cyst|Cystic]] architecture


*Gemistocytic astrocytoma is almost always [[supratentorial]] and usually located in the [[frontal lobes]].
*Gemistocytic astrocytoma is almost always [[supratentorial]] and usually located in the [[frontal lobes]].
*Infiltrative [[tumor]] with [[gemistocytes]], characterized by ample [[eosinophilic]] glassy [[cytoplasm]] and eccentric [[Cell nucleus|nuclei]] with frequent prominent [[Nucleolus|nucleoli]].
*Background is coarse fibrillary; perivascular lymphocyte cuffing is common.


*On microscopic histopathological analysis, gemistocytic astrocytoma is characterized by:<ref>{{cite book | last = Prayson | first = Richard | title = Brain tumors | publisher = Demos Medical Pub | location = New York | year = 2010 | isbn = 1933864699 }}</ref>
*On [[microscopic]] [[histopathological]] [[analysis]], gemistocytic astrocytoma is characterized by:<ref>{{cite book | last = Prayson | first = Richard | title = Brain tumors | publisher = Demos Medical Pub | location = New York | year = 2010 | isbn = 1933864699 }}</ref>


:*Neoplastic fibrillary astrocytes embedded in the tumor matrix
:*[[Cancer|Neoplastic]] fibrillary [[Astrocyte|astrocytes]] embedded in the [[tumor]] [[matrix]]
:*Gemistocytes > 20% of the tumor cells
:*[[Gemistocytes]] > 20% of the [[Tumor cell|tumor cells]]
::*Large, plump astrocytes
::*Large, plump [[Astrocyte|astrocytes]]
::*Abundant eosinophilic cytoplasm
::*Abundant [[eosinophilic]] [[cytoplasm]]
::*Eccentric nuclei
::*Eccentric [[Cell nucleus|nuclei]]
:*Low cellular density
:*Low [[Cell (biology)|cellular]] density
:*[[atypia|Mild nuclear atypia]] (enlarged, irregular contour, hyperchromasia, and coarsened nuclear chromatin pattern)
:*[[atypia|Mild nuclear atypia]] (enlarged, irregular contour, hyperchromasia, and coarsened [[Cell nucleus|nuclear]] [[chromatin]] pattern)
:*Mucinous fluid containing microcystic spaces  
:*[[Mucinous]] [[fluid]] containing microcystic spaces
:*Perivascular lymphocytic infiltrate
:*Perivascular [[Lymphocyte|lymphocytic]] infiltrate
:*Abscence of [[mitoses]], microvascular proliferation, and [[necrosis]]
:*Absence of [[mitoses]], microvascular proliferation, and [[necrosis]]


*Gemistocytic astrocytoma is demonstrated by positivity to tumor marker such as [[GFAP]].
*[[GFAP]] ([[tumor marker]]) is positive in gemistocytic astrocytoma.


==Differentiating Gemistocytic Astrocytoma from other Diseases==
==Differentiating Gemistocytic Astrocytoma from Other Diseases==
*It is important to note that other gliomas (e.g. fibrillary astrocytoma and oligodendrogliomas) can have occasional gemistocytes, without being designated a gemistocytic astrocytoma.  
*It is important to note that other [[Glioma|gliomas]] (e.g. [[fibrillary astrocytoma]] and [[Oligodendroglioma|oligodendrogliomas]]) can have occasional [[gemistocytes]], without being designated a gemistocytic astrocytoma.
*We use a cut off of 20% of the tumor cells being gemistocytes before designating it as a gemistocytic astrocytoma.<ref>{{cite book | last = Tonn | first = FirstName | title = Neuro-oncology of CNS tumors | publisher = Springer | location = Berlin New York | year = 2006 | isbn = 3540258337 }}</ref>
*A cut off of 20% of the [[Tumor cell|tumor cells]] being [[gemistocytes]], is used before designating a [[lesion]] to be gemistocytic astrocytoma.<ref>{{cite book | last = Tonn | first = FirstName | title = Neuro-oncology of CNS tumors | publisher = Springer | location = Berlin New York | year = 2006 | isbn = 3540258337 }}</ref>
*Gemistocytic astrocytoma must be differentiated from:
*Gemistocytic astrocytoma must be differentiated from:


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==Epidemiology and Demographics==
==Epidemiology and Demographics==


*Gemistocytic astrocytoma is a rare disease that tends to affect children and young adults.
*Gemistocytic astrocytoma is a rare [[disease]] that tends to affects children and young [[Adult|adults]].
*The mean age at diagnosis is 35 years.
*The [[mean]] age at [[diagnosis]] is 35 years.


*Males are more commonly affected with gemistocytic astrocytoma than females. The male to female ratio is approximately 1.5 to 1.
*[[Male|Males]] are more commonly affected than [[Female|females]]. The [[male]] to [[female]] ratio is approximately 1.5 to 1.
*Gemistocytic astrocytoma makes up approximately 10% of all WHO grade II diffuse astrocytomas. <ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>
*Gemistocytic astrocytoma makes up approximately 10% of all [[World Health Organization|WHO]] [[Grading (tumors)|grade]] II diffuse astrocytomas. <ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>


== Risk Factors ==
== Risk Factors ==
There are no specific risk factors for gemistocytic astrocytoma. For more information on risk factors for astrocytoma, [[Astrocytoma risk factors|click here]].
 
* There are no specific [[Risk factor|risk factors]] for gemistocytic astrocytoma.  
* For more information on [[Risk factor|risk factors]] for [[astrocytoma]], [[Astrocytoma risk factors|click here]].


== Screening ==
== Screening ==
There is insufficient evidence to recommend routine screening for gemcistocytic astrocytoma.
There is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for gemcistocytic astrocytoma.


==Natural History, Complications and Prognosis==
==Natural History, Complications, and Prognosis==


*If left untreated, patients with gemistocytic astrocytoma may progress to develop [[seizures]], focal neurological deficits, [[hydrocephalus]], or malignant transformation to [[anaplastic astrocytoma]] or [[glioblastoma multiforme]].
*If left untreated, [[Patient|patients]] with gemistocytic astrocytoma may progress to develop [[seizures]], focal [[Neurology|neurological]] deficits, [[hydrocephalus]], or [[malignant transformation]] to [[anaplastic astrocytoma]] or [[glioblastoma multiforme]].
*Gemistocytic astrocytoma is a slow growing tumor, but it behaves in an aggressive manner.<ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>
*Gemistocytic astrocytoma is a slow growing [[tumor]], but it behaves in an aggressive manner.<ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>


*Common complications of gemistocytic astrocytoma include:
*Common [[Complication (medicine)|complications]] of gemistocytic astrocytoma include:


:*[[Hydrocephalus]]
:*[[Hydrocephalus]]
:*Malignant transformation to [[anaplastic astrocytoma]] or [[glioblastoma multiforme]].
:*[[Malignant transformation]] to [[anaplastic astrocytoma]] or [[glioblastoma multiforme]].


*Gemistocytic astrocytoma has a poorer prognosis than the other matched WHO grade II (low-grade) astrocytic tumors ([[fibrillary astrocytoma]], protoplasmic astrocytoma, and [[oligoastrocytoma]]).
*Gemistocytic astrocytoma has a poorer [[prognosis]] than the other matched [[World Health Organization|WHO]] [[Grading (tumors)|grade]] II (low-[[Grading (tumors)|grade]]) [[Astrocyte|astrocytic]] [[Tumor|tumors]] ([[fibrillary astrocytoma]], [[protoplasmic astrocytoma]], and [[oligoastrocytoma]]).
*Gemistocytic astrocytoma has been associated with early progression and less favorable outcome; however, it remains to be determined whether the prognosis of IDH-mutant gemistocytic astrocytoma differs significantly from that of IDH-mutant diffuse astrocytoma.
*Gemistocytic astrocytoma has been associated with an early progression and less favorable outcome; however, it remains to be determined whether the [[prognosis]] of IDH-[[mutant]] gemistocytic astrocytoma differs significantly from that of IDH-[[mutant]] diffuse [[astrocytoma]].
*The 5-year survival rate of patients with gemistocytic astrocytoma is approximately 30%.<ref>{{cite book | last = Raghavan | first = Derek | title = Textbook of uncommon cancer | publisher = Wiley | location = Chichester, England | year = 2006 | isbn = 0470012021 }}</ref>
*The 5-year survival rate of [[Patient|patients]] with gemistocytic astrocytoma is approximately 30%.<ref>{{cite book | last = Raghavan | first = Derek | title = Textbook of uncommon cancer | publisher = Wiley | location = Chichester, England | year = 2006 | isbn = 0470012021 }}</ref>
*The median survival time with treatment is only 2.5 years.
*The [[median]] survival time with treatment is only 2.5 years.
*Favorable prognostic factors for gemistocytic astrocytoma include:<ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>
*The authors conclude that the presence of at least 20% gemistocytes in a [[glial neoplasm]] is a poor [[Prognosis|prognostic]] sign, irrespective of the [[pathological]] background.
* It is proposed that gemistocytic astrocytomas be [[Classification|classified]] with [[Anaplastic astrocytoma|anaplastic astrocytomas]] and treated accordingly.
* A poor [[Prognosis|prognostic]] factor for gemistocytic astrocytoma is the presence of at least 20% [[gemistocytes]] in a [[glial neoplasm]] irrespective of its [[pathological]] background.<ref name="pmid19939052">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }}</ref>
*Favorable [[Prognosis|prognostic]] factors for gemistocytic astrocytoma include:<ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>


:*Age < 50 years
:*Age < 50 years
:*Occurrence of [[seizures]] as the initial symptom
:*Occurrence of [[seizures]] as the initial [[symptom]]
:*Pre-operative symptoms lasting more than 6 months
:*Pre-operative [[symptoms]] lasting more than 6 months
:* The authors conclude that the presence of at least 20% gemistocytes in a glial neoplasm is a poor prognostic sign, irrespective of the pathological background. It is proposed that gemistocytic astrocytomas be classified with anaplastic astrocytomas and treated accordingly.
:*A poor prognostic factor for gemistocytic astrocytoma is the presence of at least 20% gemistocytes in a glial neoplasm irrespective of its pathological background.<ref name="pmid19939052">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }}</ref>


==Diagnosis==
==Diagnosis==


=== Diagnostic Study of Choice ===
=== Diagnostic Study of Choice ===
* The [[diagnosis]] of gemistocytic astrocytoma is based on the imaging findings.
* [[Magnetic resonance imaging|MRI]] and [[Computed tomography|CT scan]] of the [[brain]] is the preferred [[Diagnosis|diagnostic]] approach.


===History and Symptoms===
===History and Symptoms===


*When evaluating a patient for gemistocytic astrocytoma, 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.
*When evaluating a [[patient]] for gemistocytic astrocytoma, you should take a detailed history of the presenting [[Symptom|symptoms]] (onset, duration, and progression), other associated [[Symptom|symptoms]], and a thorough family and past [[medical]] history review.
*There are no specific factors in the [[Patient|patient’s]] history that are [[pathognomonic]] for low-[[Grading (tumors)|grade]] [[glioma]].
*The history, however, should alert the [[clinician]] to the presence of a [[Neurological disorders|neurological disorder]] and the need for an [[imaging]] study.
*Characteristically, low-[[Grading (tumors)|grade]] [[Glioma|gliomas]] present with [[headache]], focal deficit and/or, most notably [[Seizure|seizures]].<ref name="pmid17982433">{{cite journal| author=Pouratian N, Asthagiri A, Jagannathan J, Shaffrey ME, Schiff D| title=Surgery Insight: the role of surgery in the management of low-grade gliomas. | journal=Nat Clin Pract Neurol | year= 2007 | volume= 3 | issue= 11 | pages= 628-39 | pmid=17982433 | doi=10.1038/ncpneuro0634 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17982433  }}</ref>


*Symptoms of gemistocytic astrocytoma include:<ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>
*[[Symptom|Symptoms]] of gemistocytic astrocytoma include:<ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>


:*[[Headaches]]
:*[[Headaches]]
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=== Physical Examination ===
=== Physical Examination ===
There are no specific [[physical examination]] findings associated with gemistocytic astrocytoma.


=== Laboratory Findings ===
=== Laboratory Findings ===
There are no specific [[Medical laboratory|laboratory]] findings associated with gemistocytic astrocytoma.


=== Electrocardiogram ===
=== Electrocardiogram ===
There are no ECG findings associated with gemistocytic astrocytoma.
There are no [[The electrocardiogram|ECG]] findings associated with gemistocytic astrocytoma.


=== X-ray ===
=== X-ray ===
There are no x-ray findings associated with gemistocytic astrocytoma.
There are no [[X-rays|x-ray]] findings associated with gemistocytic astrocytoma.


=== Echocardiography or Ultrasound ===
=== Echocardiography or Ultrasound ===
There are no echocardiography/ultrasound findings associated with gemistocytic astrocytoma.
There are no [[echocardiography]]/[[ultrasound]] findings associated with gemistocytic astrocytoma.


===CT Scan===
===CT scan===


*Head CT scan is helpful in the diagnosis of gemistocytic astrocytoma. On CT scan, gemistocytic astrocytoma is characterized by:
*[[Head]] [[Computed tomography|CT scan]] is helpful in the [[diagnosis]] of gemistocytic astrocytoma. On [[Computed tomography|CT scan]], gemistocytic astrocytoma is characterized by:


:*Isodense or hypodense mass  
:*Iso-dense or hypo-dense mass
:*Positive mass effect
:*Positive [[Mass effect (medicine)|mass effect]]
:*Wispy enhancement (most low-grade astrocytomas are without any enhancement)
:*Wispy enhancement (most low-[[Grading (tumors)|grade]] astrocytomas are without any enhancement)
::*In fact, presence of enhancement would suggest more aggressive tumors
::*In fact, presence of enhancement would suggest more aggressive [[tumors]]
:*Calcification in 10-20% (more common in mixed tumors relating to an oligodendroglial components, i.e. [[oligoastrocytoma]])
:*[[Calcification]] in 10 - 20% (more common in mixed [[Tumor|tumors]] relating to an oligodendroglial components, i.e. [[oligoastrocytoma]])
:*Cystic or fluid attenuation components
:*[[Cyst|Cystic]] or [[fluid]] attenuation components


===MRI===
===MRI===


*Brain MRI is helpful in the diagnosis of gemistocytic astrocytoma. On MRI, gemistocytic astrocytoma is characterized by:
*[[Brain]] [[Magnetic resonance imaging|MRI]] is helpful in the [[diagnosis]] of gemistocytic astrocytoma.  
*On [[Magnetic resonance imaging|MRI]], gemistocytic astrocytoma is characterized by:


{| style="border: 0px; font-size: 90%; margin: 3px; width:1000px"
{| style="border: 0px; font-size: 90%; margin: 3px; width:1000px"
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T1
T1
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*Isointense to hypointense compared to white matter
*Iso-intense to hypo-intense compared to [[white matter]]
*Usually confined to the white matter and causes expansion of the adjacent cortex
*Usually confined to the [[white matter]] and causes expansion of the adjacent [[cortex]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
T2
T2
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*Hyperintense compared to white matter
*Hyper-intense compared to [[white matter]]
*Always follow the white matter distribution and cause expansion of the surrounding cortex
*Always follows the [[white matter]] distribution and causes expansion of the surrounding [[cortex]]
*Cortex may also be involved in late cases, in comparison to the [[oligodendroglioma]], which is a cortical based tumor from the start
*[[Cortex]] may also be involved in late cases, in comparison to the [[oligodendroglioma]], which is a [[Cortical area|cortical]] based [[tumor]] from the start
*"Microcystic changes" along the lines of spread of the infiltrative astrocytoma is a very unique behavior for the infiltrative astrocytoma, however, it is only appreciated in a few number of cases
*"Microcystic changes" along the lines of spread of the infiltrative astrocytoma is a very unique behavior for the infiltrative astrocytoma, however, it is only appreciated in a few number of cases
*Hyperintense T2 signal is not related to cellularity or cellular atypia, but rather edema, demyelination, and other degenerative changes
*Hyper-intense T2 signal is not related to [[Cell (biology)|cellularity]] or [[Cell (biology)|cellular]] [[atypia]], but rather [[edema]], [[demyelination]], and other degenerative changes
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
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| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*No enhancement
*No enhancement
*Small ill-defined areas of enhancement are not rare; however, when enhancement is seen, it should be considered as a warning sign for progression to a higher grade
*Small ill-defined areas of enhancement are not rare; however, when enhancement is seen, it should be considered as a warning sign for progression to a higher [[Grading (tumors)|grade]]
|-
|-
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
| style="padding: 5px 5px; background: #DCDCDC;font-weight: bold" align=center|
Diffusion weighted imaging (DWI)
Diffusion weighted imaging (DWI)
| style="padding: 5px 5px; background: #F5F5F5;" |
| style="padding: 5px 5px; background: #F5F5F5;" |
*No restricted diffusion
*No restricted [[diffusion]]
*Increased diffusibility is the key to differentiate the gemistocytic astrocytoma from the acute ischemia
*Increased [[Diffusion|diffusibility]] is the key to differentiate gemistocytic astrocytoma from [[Acute (medicine)|acute]] [[ischemia]]
|}
|}


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====Magnetic Resonance Spectroscopy====
====Magnetic Resonance Spectroscopy====


*[[Nuclear magnetic resonance spectroscopy|MR spectroscopy]] may be helpful in the diagnosis of gemistocytic astrocytoma.
*[[Nuclear magnetic resonance spectroscopy|MR spectroscopy]] may be helpful in the [[diagnosis]] of gemistocytic astrocytoma.
*Findings on [[Nuclear magnetic resonance spectroscopy|MR spectroscopy]] include:
*Findings on [[Nuclear magnetic resonance spectroscopy|MR spectroscopy]] include:


:*Elevated choline peak, low [[N-Acetylaspartate]] peak, elevated [[choline]]:[[creatine]] ratio
:*Elevated choline peak, low [[N-Acetylaspartate]] peak, elevated [[choline]]:[[creatine]] ratio
:*Elevated myo-inositol (mI) and mI/creatine ratio
:*Elevated myo-inositol (mI) and mI/[[creatine]] ratio
:*Lack of the lactate peak seen at 1:33
:*Lack of the [[Lactic acid|lactate]] peak seen at 1:33
::*Lactate peak represents the [[necrosis]] seen in aggressive tumors (WHO grade IV)
::*[[Lactic acid|Lactate]] peak represents the [[necrosis]] seen in aggressive [[Tumor|tumors]] ([[World Health Organization|WHO]] [[Grading (tumors)|grade]] IV)


====Magnetic Resonance Perfusion====
====Magnetic Resonance Perfusion====


*[[Perfusion weighted imaging|MR perfusion]] may be helpful in the diagnosis of gemistocytic astrocytoma, which demonstrates no elevation of relative cerebral blood volume (rCBV).
*[[Perfusion weighted imaging|MR perfusion]] may be helpful in the [[diagnosis]] of gemistocytic astrocytoma, which demonstrates no elevation of relative cerebral blood volume (rCBV).


===Other Diagnostic Studies===
===Other Diagnostic Studies===


*[[Biopsy]] of the gemistocytic astrocytoma tumor, taken through a needle during a simple surgical procedure, helps to confirm the diagnosis.
*[[Biopsy]] of the gemistocytic astrocytoma, taken via [[needle]] during a simple [[surgical procedure]], helps to confirm the [[diagnosis]].


==Treatment==
==Treatment==


=== Medical Therapy ===
=== Medical Therapy ===
Chemotherapy is recommended among patients with recurrent or de-differentiated gemistocytic astrocytomas.
[[Chemotherapy]] is recommended for [[Patient|patients]] with recurrent or de-differentiated gemistocytic astrocytomas.
<br />
=== Surgery ===
=== Surgery ===


*The predominant therapy for gemistocytic astrocytoma is surgical resection. Adjunctive [[radiation]] and nitrosourea-based [[chemotherapy]] may be required.<ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>
*The predominant [[therapy]] for gemistocytic astrocytoma is [[Resection|surgical resection]]. Adjunctive [[radiation]] and [[nitrosourea]]-based [[chemotherapy]] may be required.<ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>
*'''Surgery''': Since gemistocytic astrocytoma can behave aggressively, [[surgery]] is the mainstay of treatment.
*'''Surgery:''' Since gemistocytic astrocytoma can behave aggressively, [[surgery]] is the mainstay of treatment.
*'''Radiotherapy''': [[Radiotherapy]] may be used in gemistocytic astrocytoma post-operatively or at the time of recurrence or progression.<ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>
*'''Radiotherapy:''' [[Radiotherapy]] may be used in gemistocytic astrocytoma post-operatively or at the time of recurrence or progression.<ref name="pmid1993905">{{cite journal| author=Krouwer HG, Davis RL, Silver P, Prados M| title=Gemistocytic astrocytomas: a reappraisal. | journal=J Neurosurg | year= 1991 | volume= 74 | issue= 3 | pages= 399-406 | pmid=1993905 | doi=10.3171/jns.1991.74.3.0399 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1993905  }} </ref>
*'''Chemotherapy''': [[Chemotherapy]] may have a role in recurrent and de-differentiated tumors.
*'''Chemotherapy:''' [[Chemotherapy]] may have a role in recurrent and de-differentiated [[Tumor|tumors]].
 
=== Primary Prevention ===
There are no measures for the primary prevention of gemistocytic astrocytoma.


=== Secondary Prevention ===
There are no measures for the secondary prevention of gemiistocytic astrocytoma.


==References==
==References==

Latest revision as of 19:32, 26 August 2019

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]Roukoz A. Karam, M.D.[3]

Synonyms and keywords: Gemistocytic astrocytomas; Diffuse astrocytoma; Low grade astrocytoma

Overview

Gemistocytic astrocytoma is a histologic subtype of low grade astrocytoma, with a poorer prognosis than other matched WHO grade II astrocytic tumors. Gemistocytic astrocytoma is characterized by a significant gemistocytes population, which are large astrocytes with their cytoplasm filled with eosinophilic material displacing the nucleus eccentrically.

Historical Perspective

  • Gemistocytic astrocyte was first described by Franz Nissl in the 20th century.[1]

Classification

Gemistocytic astrocytoma is a subtype of astrocytoma and is included in the classification of astrocytoma. For more information about the classification of astrocytoma, click here.

Pathophysiology

  • Gray-tan mass
  • Well-defined borders
  • Soft texture
  • Cystic architecture

Differentiating Gemistocytic Astrocytoma from Other Diseases

Epidemiology and Demographics

  • Gemistocytic astrocytoma is a rare disease that tends to affects children and young adults.
  • The mean age at diagnosis is 35 years.
  • Males are more commonly affected than females. The male to female ratio is approximately 1.5 to 1.
  • Gemistocytic astrocytoma makes up approximately 10% of all WHO grade II diffuse astrocytomas. [5]

Risk Factors

Screening

There is insufficient evidence to recommend routine screening for gemcistocytic astrocytoma.

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

There are no specific physical examination findings associated with gemistocytic astrocytoma.

Laboratory Findings

There are no specific laboratory findings associated with gemistocytic astrocytoma.

Electrocardiogram

There are no ECG findings associated with gemistocytic astrocytoma.

X-ray

There are no x-ray findings associated with gemistocytic astrocytoma.

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with gemistocytic astrocytoma.

CT scan

  • Iso-dense or hypo-dense mass
  • Positive mass effect
  • Wispy enhancement (most low-grade astrocytomas are without any enhancement)
  • In fact, presence of enhancement would suggest more aggressive tumors

MRI

  • Brain MRI is helpful in the diagnosis of gemistocytic astrocytoma.
  • On MRI, gemistocytic astrocytoma is characterized by:
MRI component Findings

T1

T2

  • Hyper-intense compared to white matter
  • Always follows the white matter distribution and causes expansion of the surrounding cortex
  • Cortex may also be involved in late cases, in comparison to the oligodendroglioma, which is a cortical based tumor from the start
  • "Microcystic changes" along the lines of spread of the infiltrative astrocytoma is a very unique behavior for the infiltrative astrocytoma, however, it is only appreciated in a few number of cases
  • Hyper-intense T2 signal is not related to cellularity or cellular atypia, but rather edema, demyelination, and other degenerative changes

T1 with contrast

  • No enhancement
  • Small ill-defined areas of enhancement are not rare; however, when enhancement is seen, it should be considered as a warning sign for progression to a higher grade

Diffusion weighted imaging (DWI)

Other Imaging Findings

Magnetic Resonance Spectroscopy

Magnetic Resonance Perfusion

  • MR perfusion may be helpful in the diagnosis of gemistocytic astrocytoma, which demonstrates no elevation of relative cerebral blood volume (rCBV).

Other Diagnostic Studies

Treatment

Medical Therapy

Chemotherapy is recommended for patients with recurrent or de-differentiated gemistocytic astrocytomas.

Surgery

Primary Prevention

There are no measures for the primary prevention of gemistocytic astrocytoma.

Secondary Prevention

There are no measures for the secondary prevention of gemiistocytic astrocytoma.

References

  1. 1.0 1.1 1.2 Tihan, Tarik; Vohra, Poonam; Berger, Mitchel S.; Keles, G. Evren (2005). "Definition and Diagnostic Implications of Gemistocytic Astrocytomas: A Pathological Perspective". Journal of Neuro-Oncology. 76 (2): 175–183. doi:10.1007/s11060-005-4897-2. ISSN 0167-594X.
  2. Prayson, Richard (2010). Brain tumors. New York: Demos Medical Pub. ISBN 1933864699.
  3. Prayson, Richard (2010). Brain tumors. New York: Demos Medical Pub. ISBN 1933864699.
  4. Tonn, FirstName (2006). Neuro-oncology of CNS tumors. Berlin New York: Springer. ISBN 3540258337.
  5. 5.0 5.1 5.2 5.3 5.4 5.5 Krouwer HG, Davis RL, Silver P, Prados M (1991). "Gemistocytic astrocytomas: a reappraisal". J Neurosurg. 74 (3): 399–406. doi:10.3171/jns.1991.74.3.0399. PMID 1993905.
  6. Raghavan, Derek (2006). Textbook of uncommon cancer. Chichester, England: Wiley. ISBN 0470012021.
  7. Krouwer HG, Davis RL, Silver P, Prados M (1991). "Gemistocytic astrocytomas: a reappraisal". J Neurosurg. 74 (3): 399–406. doi:10.3171/jns.1991.74.3.0399. PMID 1993905.
  8. Pouratian N, Asthagiri A, Jagannathan J, Shaffrey ME, Schiff D (2007). "Surgery Insight: the role of surgery in the management of low-grade gliomas". Nat Clin Pract Neurol. 3 (11): 628–39. doi:10.1038/ncpneuro0634. PMID 17982433.

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