Gliomatosis cerebri pathophysiology: Difference between revisions

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==Overview==
==Overview==
Genes involved in pathogenesis of gliomatosis cerebri include ''[[p53]]'', ''[[OLIG2|OLIG-2]]'', ''[[Ki-67 (Biology)|Ki-67]]'', ''[[EGFR]]'', ''[[PTEN]]'', ''[[VCAM1]]'', ''[[VEGF]]'', and gene on chromosomes [[Chromosome 7|7q]], [[Chromosome 10|10q]], and [[Chromosome 13|13q]].<ref name="pmid20223351">{{cite journal| author=San Millan B, Kaci R, Polivka M, Robert G, Héran F, Gueguen A et al.| title=[Gliomatosis cerebri: a biopsy and autopsy case report]. | journal=Ann Pathol | year= 2010 | volume= 30 | issue= 1 | pages= 25-9 | pmid=20223351 | doi=10.1016/j.annpat.2009.10.020 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20223351  }} </ref><ref name="pmid17228264">{{cite journal| author=Ware ML, Hirose Y, Scheithauer BW, Yeh RF, Mayo MC, Smith JS et al.| title=Genetic aberrations in gliomatosis cerebri. | journal=Neurosurgery | year= 2007 | volume= 60 | issue= 1 | pages= 150-8; discussion 158 | pmid=17228264 | doi=10.1227/01.NEU.0000249203.73849.5D | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17228264  }} </ref><ref name="pmid12325066">{{cite journal| author=Herrlinger U, Felsberg J, Küker W, Bornemann A, Plasswilm L, Knobbe CB et al.| title=Gliomatosis cerebri: molecular pathology and clinical course. | journal=Ann Neurol | year= 2002 | volume= 52 | issue= 4 | pages= 390-9 | pmid=12325066 | doi=10.1002/ana.10297 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12325066  }} </ref><ref name="pmid19830138">{{cite journal| author=D'Urso PI, D'Urso OF, Marsigliante S, Storelli C, Distante A, Sanguedolce F et al.| title=Gliomatosis cerebri type II: two case reports. | journal=J Med Case Rep | year= 2009 | volume= 3 | issue=  | pages= 7225 | pmid=19830138 | doi=10.4076/1752-1947-3-7225 | pmc=PMC2726545 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19830138  }} </ref> Gliomatosis cerebri may be associated with [[neurofibromatosis type 1]] and [[pilomatricoma]].<ref name="pmid21837541">{{cite journal| author=Buis DR, van der Valk P, De Witt Hamer PC| title=Subcutaneous tumor seeding after biopsy in gliomatosis cerebri. | journal=J Neurooncol | year= 2012 | volume= 106 | issue= 2 | pages= 431-5 | pmid=21837541 | doi=10.1007/s11060-011-0678-2 | pmc=PMC3230756 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21837541  }} </ref><ref name="pmid19250412">{{cite journal| author=Wachter-Giner T, Bieber I, Warmuth-Metz M, Bröcker EB, Hamm H| title=Multiple pilomatricomas and gliomatosis cerebri--a new association? | journal=Pediatr Dermatol | year= 2009 | volume= 26 | issue= 1 | pages= 75-8 | pmid=19250412 | doi=10.1111/j.1525-1470.2008.00827.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19250412  }} </ref> On gross pathology, gliomatosis cerebri is characterized by a diffuse [[astrocytic]] growth pattern involving at least three cerebral lobes and bilateral involvement of the [[cerebrum|cerebral hemispheres]], [[gray matter|deep gray matter]], [[brainstem]], or [[cerebellum]].<ref name="pmid12325066">{{cite journal| author=Herrlinger U, Felsberg J, Küker W, Bornemann A, Plasswilm L, Knobbe CB et al.| title=Gliomatosis cerebri: molecular pathology and clinical course. | journal=Ann Neurol | year= 2002 | volume= 52 | issue= 4 | pages= 390-9 | pmid=12325066 | doi=10.1002/ana.10297 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12325066  }} </ref><ref name="pmid21837541">{{cite journal| author=Buis DR, van der Valk P, De Witt Hamer PC| title=Subcutaneous tumor seeding after biopsy in gliomatosis cerebri. | journal=J Neurooncol | year= 2012 | volume= 106 | issue= 2 | pages= 431-5 | pmid=21837541 | doi=10.1007/s11060-011-0678-2 | pmc=PMC3230756 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21837541  }} </ref><ref name=librepath>Gliomatosis cerebri. Libre pathology. http://librepathology.org/wiki/index.php/Astrocytoma#Gliomatosis_cerebri</ref> On microscopic histopathological examination, gliomatosis cerebri is characterized by diffuse proliferation of immature glial elements resembling [[astrocytes]], [[oligodendroglia]], or undifferentiated cells with [[Atypia|cytologic and nuclear atypia]], [[calcification]]s, microcysts, and [[Mitoses|mitotic figures]] without the presence of any [[necrosis]] or microvascular proliferation.<ref name="pmid4053456">{{cite journal| author=Artigas J, Cervos-Navarro J, Iglesias JR, Ebhardt G| title=Gliomatosis cerebri: clinical and histological findings. | journal=Clin Neuropathol | year= 1985 | volume= 4 | issue= 4 | pages= 135-48 | pmid=4053456 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4053456  }} </ref> Gliomatosis cerebri is demonstrated by positivity to [[tumor markers]] such as [[GFAP]], [[S-100 protein|S-100]], and [[Ki-67 (Biology)|Ki-67]].<ref name="pmid8218127">{{cite journal| author=Galatioto S, Marafioti T, Cavallari V, Batolo D| title=Gliomatosis cerebri. Clinical, neuropathological, immunohistochemical and morphometric Studies. | journal=Zentralbl Pathol | year= 1993 | volume= 139 | issue= 3 | pages= 261-7 | pmid=8218127 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8218127  }} </ref><ref name="pmid19353837">{{cite journal| author=Park S, Suh YL, Nam DH, Kim ST| title=Gliomatosis cerebri: clinicopathologic study of 33 cases and comparison of mass forming and diffuse types. | journal=Clin Neuropathol | year= 2009 | volume= 28 | issue= 2 | pages= 73-82 | pmid=19353837 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19353837  }} </ref>
Genes involved in pathogenesis of gliomatosis cerebri include ''[[p53]]'', ''[[OLIG2|OLIG-2]]'', ''[[Ki-67 (Biology)|Ki-67]]'', ''[[EGFR]]'', ''[[PTEN]]'', ''[[VCAM1]]'', ''[[VEGF]]'', and gene on chromosomes [[Chromosome 7|7q]], [[Chromosome 10|10q]], and [[Chromosome 13|13q]].<ref name="pmid20223351">{{cite journal| author=San Millan B, Kaci R, Polivka M, Robert G, Héran F, Gueguen A et al.| title=[Gliomatosis cerebri: a biopsy and autopsy case report]. | journal=Ann Pathol | year= 2010 | volume= 30 | issue= 1 | pages= 25-9 | pmid=20223351 | doi=10.1016/j.annpat.2009.10.020 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20223351  }} </ref><ref name="pmid17228264">{{cite journal| author=Ware ML, Hirose Y, Scheithauer BW, Yeh RF, Mayo MC, Smith JS et al.| title=Genetic aberrations in gliomatosis cerebri. | journal=Neurosurgery | year= 2007 | volume= 60 | issue= 1 | pages= 150-8; discussion 158 | pmid=17228264 | doi=10.1227/01.NEU.0000249203.73849.5D | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=17228264  }} </ref><ref name="pmid12325066">{{cite journal| author=Herrlinger U, Felsberg J, Küker W, Bornemann A, Plasswilm L, Knobbe CB et al.| title=Gliomatosis cerebri: molecular pathology and clinical course. | journal=Ann Neurol | year= 2002 | volume= 52 | issue= 4 | pages= 390-9 | pmid=12325066 | doi=10.1002/ana.10297 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12325066  }} </ref><ref name="pmid19830138">{{cite journal| author=D'Urso PI, D'Urso OF, Marsigliante S, Storelli C, Distante A, Sanguedolce F et al.| title=Gliomatosis cerebri type II: two case reports. | journal=J Med Case Rep | year= 2009 | volume= 3 | issue=  | pages= 7225 | pmid=19830138 | doi=10.4076/1752-1947-3-7225 | pmc=PMC2726545 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19830138  }} </ref> Gliomatosis cerebri may be associated with [[neurofibromatosis type 1]] and [[pilomatricoma]].<ref name="pmid21837541">{{cite journal| author=Buis DR, van der Valk P, De Witt Hamer PC| title=Subcutaneous tumor seeding after biopsy in gliomatosis cerebri. | journal=J Neurooncol | year= 2012 | volume= 106 | issue= 2 | pages= 431-5 | pmid=21837541 | doi=10.1007/s11060-011-0678-2 | pmc=PMC3230756 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21837541  }} </ref><ref name="pmid19250412">{{cite journal| author=Wachter-Giner T, Bieber I, Warmuth-Metz M, Bröcker EB, Hamm H| title=Multiple pilomatricomas and gliomatosis cerebri--a new association? | journal=Pediatr Dermatol | year= 2009 | volume= 26 | issue= 1 | pages= 75-8 | pmid=19250412 | doi=10.1111/j.1525-1470.2008.00827.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19250412  }} </ref> On gross pathology, gliomatosis cerebri is characterized by a diffuse [[astrocytic]] growth pattern involving at least three cerebral lobes and bilateral involvement of the [[cerebrum|cerebral hemispheres]], [[gray matter|deep gray matter]], [[brainstem]], or [[cerebellum]].<ref name="pmid12325066">{{cite journal| author=Herrlinger U, Felsberg J, Küker W, Bornemann A, Plasswilm L, Knobbe CB et al.| title=Gliomatosis cerebri: molecular pathology and clinical course. | journal=Ann Neurol | year= 2002 | volume= 52 | issue= 4 | pages= 390-9 | pmid=12325066 | doi=10.1002/ana.10297 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12325066  }} </ref><ref name="pmid21837541">{{cite journal| author=Buis DR, van der Valk P, De Witt Hamer PC| title=Subcutaneous tumor seeding after biopsy in gliomatosis cerebri. | journal=J Neurooncol | year= 2012 | volume= 106 | issue= 2 | pages= 431-5 | pmid=21837541 | doi=10.1007/s11060-011-0678-2 | pmc=PMC3230756 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21837541  }} </ref><ref name="librepath">Gliomatosis cerebri. Libre pathology. http://librepathology.org/wiki/index.php/Astrocytoma#Gliomatosis_cerebri</ref> On microscopic histopathological examination, gliomatosis cerebri is characterized by diffuse proliferation of immature glial elements resembling [[astrocytes]], [[oligodendroglia]], or undifferentiated cells with [[Atypia|cytologic and nuclear atypia]], [[calcification]]s, microcysts, and [[Mitoses|mitotic figures]] without the presence of any [[necrosis]] or microvascular proliferation.<ref name="pmid4053456">{{cite journal| author=Artigas J, Cervos-Navarro J, Iglesias JR, Ebhardt G| title=Gliomatosis cerebri: clinical and histological findings. | journal=Clin Neuropathol | year= 1985 | volume= 4 | issue= 4 | pages= 135-48 | pmid=4053456 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4053456  }} </ref> Gliomatosis cerebri is demonstrated by positivity to [[tumor markers]] such as [[GFAP]], [[S-100 protein|S-100]], and [[Ki-67 (Biology)|Ki-67]].<ref name="pmid8218127">{{cite journal| author=Galatioto S, Marafioti T, Cavallari V, Batolo D| title=Gliomatosis cerebri. Clinical, neuropathological, immunohistochemical and morphometric Studies. | journal=Zentralbl Pathol | year= 1993 | volume= 139 | issue= 3 | pages= 261-7 | pmid=8218127 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8218127  }} </ref><ref name="pmid19353837">{{cite journal| author=Park S, Suh YL, Nam DH, Kim ST| title=Gliomatosis cerebri: clinicopathologic study of 33 cases and comparison of mass forming and diffuse types. | journal=Clin Neuropathol | year= 2009 | volume= 28 | issue= 2 | pages= 73-82 | pmid=19353837 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19353837  }} </ref>


==Pathophysiology==
==Pathophysiology==
Line 26: Line 26:


===Gross Pathology===
===Gross Pathology===
On gross pathology, gliomatosis cerebri is characterized by:<ref name="pmid12325066">{{cite journal| author=Herrlinger U, Felsberg J, Küker W, Bornemann A, Plasswilm L, Knobbe CB et al.| title=Gliomatosis cerebri: molecular pathology and clinical course. | journal=Ann Neurol | year= 2002 | volume= 52 | issue= 4 | pages= 390-9 | pmid=12325066 | doi=10.1002/ana.10297 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12325066  }} </ref><ref name="pmid21837541">{{cite journal| author=Buis DR, van der Valk P, De Witt Hamer PC| title=Subcutaneous tumor seeding after biopsy in gliomatosis cerebri. | journal=J Neurooncol | year= 2012 | volume= 106 | issue= 2 | pages= 431-5 | pmid=21837541 | doi=10.1007/s11060-011-0678-2 | pmc=PMC3230756 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21837541  }} </ref><ref name=librepath>Gliomatosis cerebri. Libre pathology. http://librepathology.org/wiki/index.php/Astrocytoma#Gliomatosis_cerebri</ref>
On gross pathology, gliomatosis cerebri is characterized by:<ref name="pmid12325066">{{cite journal| author=Herrlinger U, Felsberg J, Küker W, Bornemann A, Plasswilm L, Knobbe CB et al.| title=Gliomatosis cerebri: molecular pathology and clinical course. | journal=Ann Neurol | year= 2002 | volume= 52 | issue= 4 | pages= 390-9 | pmid=12325066 | doi=10.1002/ana.10297 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12325066  }} </ref><ref name="pmid21837541">{{cite journal| author=Buis DR, van der Valk P, De Witt Hamer PC| title=Subcutaneous tumor seeding after biopsy in gliomatosis cerebri. | journal=J Neurooncol | year= 2012 | volume= 106 | issue= 2 | pages= 431-5 | pmid=21837541 | doi=10.1007/s11060-011-0678-2 | pmc=PMC3230756 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21837541  }} </ref><ref name="librepath">Gliomatosis cerebri. Libre pathology. http://librepathology.org/wiki/index.php/Astrocytoma#Gliomatosis_cerebri</ref>
*Diffuse, usually [[astrocytic]] growth pattern
*Diffuse, usually [[astrocytic]] growth pattern
*Involvement of at least three cerebral lobes
*Involvement of at least three cerebral lobes
Line 52: Line 52:
Gliomatosis cerebri is demonstrated by positivity to [[tumor markers]] such as [[GFAP]], [[S-100 protein|S-100]], and [[Ki-67 (Biology)|Ki-67]].<ref name="pmid8218127">{{cite journal| author=Galatioto S, Marafioti T, Cavallari V, Batolo D| title=Gliomatosis cerebri. Clinical, neuropathological, immunohistochemical and morphometric Studies. | journal=Zentralbl Pathol | year= 1993 | volume= 139 | issue= 3 | pages= 261-7 | pmid=8218127 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8218127  }} </ref><ref name="pmid19353837">{{cite journal| author=Park S, Suh YL, Nam DH, Kim ST| title=Gliomatosis cerebri: clinicopathologic study of 33 cases and comparison of mass forming and diffuse types. | journal=Clin Neuropathol | year= 2009 | volume= 28 | issue= 2 | pages= 73-82 | pmid=19353837 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19353837  }} </ref>
Gliomatosis cerebri is demonstrated by positivity to [[tumor markers]] such as [[GFAP]], [[S-100 protein|S-100]], and [[Ki-67 (Biology)|Ki-67]].<ref name="pmid8218127">{{cite journal| author=Galatioto S, Marafioti T, Cavallari V, Batolo D| title=Gliomatosis cerebri. Clinical, neuropathological, immunohistochemical and morphometric Studies. | journal=Zentralbl Pathol | year= 1993 | volume= 139 | issue= 3 | pages= 261-7 | pmid=8218127 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8218127  }} </ref><ref name="pmid19353837">{{cite journal| author=Park S, Suh YL, Nam DH, Kim ST| title=Gliomatosis cerebri: clinicopathologic study of 33 cases and comparison of mass forming and diffuse types. | journal=Clin Neuropathol | year= 2009 | volume= 28 | issue= 2 | pages= 73-82 | pmid=19353837 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19353837  }} </ref>


==Pathophysiology==
<br />
===Physiology===
The normal physiology of [name of process] can be understood as follows:
 
===Pathogenesis===
*The exact pathogenesis of [disease name] is not completely understood.
OR
*It is understood that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
*[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
*Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
*[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
*The progression to [disease name] usually involves the [molecular pathway].
*The pathophysiology of [disease/malignancy] depends on the histological subtype.
 
==Genetics==
[Disease name] is transmitted in [mode of genetic transmission] pattern.
 
OR
 
Genes involved in the pathogenesis of [disease name] include:
*[Gene1]
*[Gene2]
*[Gene3]
 
OR
 
The development of [disease name] is the result of multiple genetic mutations such as:
 
*[Mutation 1]
*[Mutation 2]
*[Mutation 3]
 
==Associated Conditions==
Conditions associated with [disease name] include:
 
*[Condition 1]
*[Condition 2]
*[Condition 3]
 
==Gross Pathology==
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
 
==Microscopic Pathology==
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].


==References==
==References==

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

Overview

Genes involved in pathogenesis of gliomatosis cerebri include p53, OLIG-2, Ki-67, EGFR, PTEN, VCAM1, VEGF, and gene on chromosomes 7q, 10q, and 13q.[1][2][3][4] Gliomatosis cerebri may be associated with neurofibromatosis type 1 and pilomatricoma.[5][6] On gross pathology, gliomatosis cerebri is characterized by a diffuse astrocytic growth pattern involving at least three cerebral lobes and bilateral involvement of the cerebral hemispheres, deep gray matter, brainstem, or cerebellum.[3][5][7] On microscopic histopathological examination, gliomatosis cerebri is characterized by diffuse proliferation of immature glial elements resembling astrocytes, oligodendroglia, or undifferentiated cells with cytologic and nuclear atypia, calcifications, microcysts, and mitotic figures without the presence of any necrosis or microvascular proliferation.[8] Gliomatosis cerebri is demonstrated by positivity to tumor markers such as GFAP, S-100, and Ki-67.[9][10]

Pathophysiology

Genetics

Genes involved in pathogenesis of gliomatosis cerebri include:[1][2][3][4]

Associated Conditions

Gliomatosis cerebri may be associated with:[11][5][6]

Gross Pathology

On gross pathology, gliomatosis cerebri is characterized by:[3][5][7]

Common intracranial sites involved in gliomatosis cerebri include:[12]

Microscopic Pathology

On microscopic histopathological examination, gliomatosis cerebri is characterized by:[8]

According to WHO classification of brain tumors, gliomatosis cerebri is classified into grade 2 or grade 3 tumors.

Immunohistochemistry

Gliomatosis cerebri is demonstrated by positivity to tumor markers such as GFAP, S-100, and Ki-67.[9][10]


References

  1. ↑ 1.0 1.1 San Millan B, Kaci R, Polivka M, Robert G, Héran F, Gueguen A; et al. (2010). "[Gliomatosis cerebri: a biopsy and autopsy case report]". Ann Pathol. 30 (1): 25–9. doi:10.1016/j.annpat.2009.10.020. PMID 20223351.
  2. ↑ 2.0 2.1 Ware ML, Hirose Y, Scheithauer BW, Yeh RF, Mayo MC, Smith JS; et al. (2007). "Genetic aberrations in gliomatosis cerebri". Neurosurgery. 60 (1): 150–8, discussion 158. doi:10.1227/01.NEU.0000249203.73849.5D. PMID 17228264.
  3. ↑ 3.0 3.1 3.2 3.3 Herrlinger U, Felsberg J, Küker W, Bornemann A, Plasswilm L, Knobbe CB; et al. (2002). "Gliomatosis cerebri: molecular pathology and clinical course". Ann Neurol. 52 (4): 390–9. doi:10.1002/ana.10297. PMID 12325066.
  4. ↑ 4.0 4.1 D'Urso PI, D'Urso OF, Marsigliante S, Storelli C, Distante A, Sanguedolce F; et al. (2009). "Gliomatosis cerebri type II: two case reports". J Med Case Rep. 3: 7225. doi:10.4076/1752-1947-3-7225. PMC 2726545. PMID 19830138.
  5. ↑ 5.0 5.1 5.2 5.3 Buis DR, van der Valk P, De Witt Hamer PC (2012). "Subcutaneous tumor seeding after biopsy in gliomatosis cerebri". J Neurooncol. 106 (2): 431–5. doi:10.1007/s11060-011-0678-2. PMC 3230756. PMID 21837541.
  6. ↑ 6.0 6.1 Wachter-Giner T, Bieber I, Warmuth-Metz M, Bröcker EB, Hamm H (2009). "Multiple pilomatricomas and gliomatosis cerebri--a new association?". Pediatr Dermatol. 26 (1): 75–8. doi:10.1111/j.1525-1470.2008.00827.x. PMID 19250412.
  7. ↑ 7.0 7.1 Gliomatosis cerebri. Libre pathology. http://librepathology.org/wiki/index.php/Astrocytoma#Gliomatosis_cerebri
  8. ↑ 8.0 8.1 Artigas J, Cervos-Navarro J, Iglesias JR, Ebhardt G (1985). "Gliomatosis cerebri: clinical and histological findings". Clin Neuropathol. 4 (4): 135–48. PMID 4053456.
  9. ↑ 9.0 9.1 Galatioto S, Marafioti T, Cavallari V, Batolo D (1993). "Gliomatosis cerebri. Clinical, neuropathological, immunohistochemical and morphometric Studies". Zentralbl Pathol. 139 (3): 261–7. PMID 8218127.
  10. ↑ 10.0 10.1 Park S, Suh YL, Nam DH, Kim ST (2009). "Gliomatosis cerebri: clinicopathologic study of 33 cases and comparison of mass forming and diffuse types". Clin Neuropathol. 28 (2): 73–82. PMID 19353837.
  11. ↑ Koszyca B, Moore L, Byard RW (1993). "Lethal manifestations of neurofibromatosis type 1 in childhood". Pediatr Pathol. 13 (5): 573–81. PMID 8247955.
  12. ↑ Brandão RA, de Carvalho GT, de Azeredo Coutinho CA, Christo PP, Santiago CF, Santos Mdo C; et al. (2011). "Gliomatosis cerebri: diagnostic considerations in three cases". Neurol India. 59 (1): 122–5. doi:10.4103/0028-3886.76892. PMID 21339680.


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