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], 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]
- P53
- Isocitrate dehydrogenase (IDH1)
- OLIG-2
- Ki-67
- EGFR
- PTEN
- VCAM1
- VEGF
- Gene on chromosome 7q
- Gene on chromosome 10q
- Gene on chromosome 13q
Associated Conditions
Gliomatosis cerebri may be associated with:[5][6][7]
Gross Pathology
On gross pathology, gliomatosis cerebri is characterized by:[3][6][8]
- Diffuse, usually astrocytic growth pattern
- Involvement of at least three lobes of the brain [9]
- Bilateral involvement of the cerebral hemispheres, deep gray matter, brainstem, or cerebellum
Common intracranial sites involved in gliomatosis cerebri include:[10]
- 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:[11]
- 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.[12][13]
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.
- ↑ Koszyca B, Moore L, Byard RW (1993). "Lethal manifestations of neurofibromatosis type 1 in childhood". Pediatr Pathol. 13 (5): 573–81. PMID 8247955.
- ↑ 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.
- ↑ 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
- ↑ "Gliomatosis cerebri: no evidence for a separate brain tumor entity".
- ↑ 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.