Gliomatosis cerebri pathophysiology: Difference between revisions

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Revision as of 15:31, 5 October 2015

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 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]

Pathophysiology

Genetics

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

Associated Conditions

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

Gross Pathology

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

Diffuse, usually astrocytic growth pattern
Involvement of at least three cerebral lobes
Bilateral involvement of the cerebral hemispheres, deep gray matter, brainstem, or cerebellum

Common intracranial sites involved by gliomatosis cerebri include:^[8]

Basal and 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:^[9]

Diffuse proliferation of immature glial elements resembling astrocytes, oligodendroglia, or undifferentiated cells
Cytologic and nuclear atypia
Calcification
Microcysts
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, and Ki-67.^[10]^[11]

References

↑ ^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.
↑ ^2.0 ^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.
↑ ^3.0 ^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.
↑ ^4.0 ^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.
↑ ^5.0 ^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.
↑ ^6.0 ^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.
↑ ^7.0 ^7.1 Gliomatosis cerebri. Libre pathology. http://librepathology.org/wiki/index.php/Astrocytoma#Gliomatosis_cerebri
↑ 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.
↑ Artigas J, Cervos-Navarro J, Iglesias JR, Ebhardt G (1985). "Gliomatosis cerebri: clinical and histological findings". Clin Neuropathol. 4 (4): 135–48. PMID 4053456.
↑ 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.
↑ 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.

Template:WikiDoc Sources

[pmid20223351-1] 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.

[pmid17228264-2] 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.

[pmid12325066-3] 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.

[pmid19830138-4] 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.

[pmid21837541-5] 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.

[pmid19250412-6] 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.

[librepath-7] 7.0 ^7.1 Gliomatosis cerebri. Libre pathology. http://librepathology.org/wiki/index.php/Astrocytoma#Gliomatosis_cerebri

[pmid21339680-8] 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.

[pmid4053456-9] Artigas J, Cervos-Navarro J, Iglesias JR, Ebhardt G (1985). "Gliomatosis cerebri: clinical and histological findings". Clin Neuropathol. 4 (4): 135–48. PMID 4053456.

[pmid8218127-10] 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.

[pmid19353837-11] 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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@@ Line 4: / Line 4: @@
 ==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>
+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 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>
 ==Pathophysiology==