Gliomatosis cerebri pathophysiology
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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
Pathophysiology
Pathogenesis
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
- ↑ 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.
- ↑ 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 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.
- ↑ 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 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.
- ↑ 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.
- ↑ 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.