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{{Gliomatosis cerebri}}
{{Gliomatosis cerebri}}
{{CMG}}{{AE}}{{SR}}, [[ nabeel ahmed ]]


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==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]]. Gliomatosis cerebri may be associated with [[neurofibromatosis type 1]] and [[pilomatricoma]]. The most common form of GC which is astrocytic involves mutation in p53 and (isocitrate dehydrogenase) IDH1. 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]]. 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. Gliomatosis cerebri is demonstrated by positivity to [[tumor markers]] such as [[GFAP]], [[S-100 protein|S-100]], and [[Ki-67 (Biology)|Ki-67]].
 
==Pathophysiology==
===Genetics===
Genes involved in pathogenesis of gliomatosis cerebri include:<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>
*''[[P53]]''
*''Isocitrate dehydrogenase (IDH1)''
*''[[OLIG2|OLIG-2]]''
*''[[Ki-67 (Biology)|Ki-67]]''
*''[[EGFR]]''
*''[[PTEN]]''
*''[[VCAM1]]''
*''[[VEGF]]''
*[[Chromosome 7|Gene on chromosome 7q]]
*[[Chromosome 10|Gene on chromosome 10q]]
*[[Chromosome 13|Gene on chromosome 13q]]
 
===Associated Conditions===
Gliomatosis cerebri may be associated with:<ref name="pmid8247955">{{cite journal| author=Koszyca B, Moore L, Byard RW| title=Lethal manifestations of neurofibromatosis type 1 in childhood. | journal=Pediatr Pathol | year= 1993 | volume= 13 | issue= 5 | pages= 573-81 | pmid=8247955 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8247955  }} </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="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>
*[[Neurofibromatosis type 1]]
*[[Pilomatricoma]]
 
===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>
*Diffuse, usually [[astrocytic]] growth pattern
*Involvement of at least three lobes of the brain <ref>{{Cite web|url=https://www.ncbi.nlm.nih.gov/pubmed/26493382|title=Gliomatosis cerebri: no evidence for a separate brain tumor entity|last=|first=|date=|website=|archive-url=|archive-date=|dead-url=|access-date=}}</ref>
*Bilateral involvement of the [[cerebrum|cerebral hemispheres]], [[gray matter|deep gray matter]], [[brainstem]], or [[cerebellum]]
 
Common intracranial sites involved in gliomatosis cerebri include:<ref name="pmid21339680">{{cite journal| author=Brandão RA, de Carvalho GT, de Azeredo Coutinho CA, Christo PP, Santiago CF, Santos Mdo C et al.| title=Gliomatosis cerebri: diagnostic considerations in three cases. | journal=Neurol India | year= 2011 | volume= 59 | issue= 1 | pages= 122-5 | pmid=21339680 | doi=10.4103/0028-3886.76892 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21339680  }} </ref>
*[[basal ganglia|Basal]] and [[thalamus|thalamic nuclei]] (75%)
*[[Corpus callosum]] (50%)
*[[Brainstem]] and [[spinal cord]] (10-15%)
*[[Cerebellum]] (10%)
**Two or more sites generally affected at the same time
 
===Microscopic Pathology===
On microscopic histopathological examination, gliomatosis cerebri is characterized by:<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>
*Diffuse proliferation of immature glial elements resembling [[astrocytes]], [[oligodendroglia]], or undifferentiated cells
*[[Atypia|Cytologic and nuclear atypia]]
*[[Calcification]]
*[[Cyst|Microcysts]]
*[[Mitoses|Mitotic figures]]
*No [[necrosis]] or microvascular proliferation
 
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 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>
 
<br />


==References==
==References==
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Latest revision as of 16:19, 7 October 2019

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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], nabeel ahmed

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. Gliomatosis cerebri may be associated with neurofibromatosis type 1 and pilomatricoma. The most common form of GC which is astrocytic involves mutation in p53 and (isocitrate dehydrogenase) IDH1. 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. 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. Gliomatosis cerebri is demonstrated by positivity to tumor markers such as GFAP, S-100, and Ki-67.

Pathophysiology

Genetics

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

Associated Conditions

Gliomatosis cerebri may be associated with:[5][6][7]

Gross Pathology

On gross pathology, gliomatosis cerebri is characterized by:[3][6][8]

Common intracranial sites involved in gliomatosis cerebri include:[10]

Microscopic Pathology

On microscopic histopathological examination, gliomatosis cerebri is characterized by:[11]

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.[12][13]


References

  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. ↑ 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 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. ↑ 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. ↑ Koszyca B, Moore L, Byard RW (1993). "Lethal manifestations of neurofibromatosis type 1 in childhood". Pediatr Pathol. 13 (5): 573–81. PMID 8247955.
  6. ↑ 6.0 6.1 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.
  7. ↑ 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.
  8. ↑ Gliomatosis cerebri. Libre pathology. http://librepathology.org/wiki/index.php/Astrocytoma#Gliomatosis_cerebri
  9. ↑ "Gliomatosis cerebri: no evidence for a separate brain tumor entity".
  10. ↑ 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.
  11. ↑ Artigas J, Cervos-Navarro J, Iglesias JR, Ebhardt G (1985). "Gliomatosis cerebri: clinical and histological findings". Clin Neuropathol. 4 (4): 135–48. PMID 4053456.
  12. ↑ 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.
  13. ↑ 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.


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