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| ==Overview== | | ==Overview== |
| Gliomatosis cerebri is a rare primary [[brain tumor]]. According to WHO, gliomatosis cerebri is classified as a distinct nosological entity among other glial tumors of the central nervous system. Gliomatosis cerebri is defined as a diffusely infiltrating neuroepithelial tumor, which involves at least three cerebral lobes and occasionally [[infratentorial]] structures or the [[spinal cord]]. The [[brain]] architecture is commonly preserved, with [[neurons]] being spared, and the mass effect is minimal. Gliomatosis cerebri was first reported by Landau in 1910. Since then, several physicians published isolated cases of gliomatosis cerebri and only later in 1938, it was completely studied and described by Nevin.<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> 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, [[cyst|microcyst]]s, 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 a rare [[brain tumor]]. The incidence of gliomatosis cerebri is estimated to be less than 100 cases per year in the United States.<ref name=epi> Gliomatosis cerebri international registry. http://gcregistry.com/</ref> Gliomatosis cerebri is a disease that tends to affect the middle-aged population. The peak incidence for gliomatosis cerebri is 20-40 years.<ref name=aaa>Epidemiology of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref> The median age at diagnosis is 34 years.<ref name="pmid9810441">{{cite journal| author=Kim DG, Yang HJ, Park IA, Chi JG, Jung HW, Han DH et al.| title=Gliomatosis cerebri: clinical features, treatment, and prognosis. | journal=Acta Neurochir (Wien) | year= 1998 | volume= 140 | issue= 8 | pages= 755-62 | pmid=9810441 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9810441 }} </ref> Males are more commonly affected with gliomatosis cerebri than females. The male to female ratio is approximately 1.5 to 1.<ref name=aaa>Epidemiology of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref> If left untreated, patients with gliomatosis cerebri may progress to develop focal neurological deficits, [[altered mental status]], [[hydrocephalus]], [[brain herniation]], and ultimately death. Transformation into glioblastoma (grade 4) may occur a few years later.<ref name=aaa>Treatment and prognosis of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref> Common complications of gliomatosis cerebri include [[brain herniation]], [[hydrocephalus]], [[coma]], [[metastasis]], recurrence, [[benign intracranial hypertension]], and side effects of [[radiotherapy]] and [[chemotherapy]].<ref name="pmid12493121">{{cite journal| author=Weinberg JS, Rhines LD, Cohen ZR, Langford L, Levin VA| title=Posterior fossa decompression for life-threatening tonsillar herniation in patients with gliomatosis cerebri: report of three cases. | journal=Neurosurgery | year= 2003 | volume= 52 | issue= 1 | pages= 216-23; discussion 223 | pmid=12493121 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12493121 }} </ref><ref name=aaa>Treatment and prognosis of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref><ref name="pmidhttp://dx.doi.org/10.1017/S0012162201000214 (About">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=http://dx.doi.org/10.1017/S0012162201000214 (About | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref><ref name="pmid3124573">{{cite journal| author=Dickson DW, Horoupian DS, Thal LJ, Lantos G| title=Gliomatosis cerebri presenting with hydrocephalus and dementia. | journal=AJNR Am J Neuroradiol | year= 1988 | volume= 9 | issue= 1 | pages= 200-2 | pmid=3124573 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3124573 }} </ref><ref name="pmid16617385">{{cite journal| author=Krutsay M, Sipos G| title=[Gliomatosis cerebri]. | journal=Magy Onkol | year= 2006 | volume= 50 | issue= 1 | pages= 55-8 | pmid=16617385 | doi=HUON.2006.50.1.0055 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16617385 }} </ref><ref name="pmid10328549">{{cite journal| author=Kannuki S, Hirose T, Horiguchi H, Kageji T, Nagahiro S| title=Gliomatosis cerebri with secondary glioblastoma formation: report of two cases. | journal=Brain Tumor Pathol | year= 1998 | volume= 15 | issue= 2 | pages= 111-6 | pmid=10328549 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10328549 }} </ref> Prognosis of gliomatosis cerebri is generally poor, and the 5-year survival rate of patients with treatment is approximately 17.7%. Symptoms of gliomatosis cerebri include [[headache]], [[nausea]], [[vomiting]], [[seizure]], [[Gait ataxia|loss of balance]], [[memory loss]], [[personality changes]], [[Cognition|cognitive problems]], [[confusion]], [[diplopia]], [[Dysphagia|difficulty in swallowing]], [[Dysarthria|difficulty in speech]], [[Weakness|motor weakness]], and [[Numbness|facial numbness]].<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> On head CT scan, gliomatosis cerebri is characterized by an isodense and hypoattenuated mass with ill-defined asymmetry and lack of mass effect. On brain MRI, gliomatosis cerebri is characterized by bilateral and diffuse infiltrative tumor which is isointense or hypointense on T1-weighted images and hyperintense on T2-weighted and FLAIR images.<ref name=aaa>Radiographic features of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref> Other imaging studies for gliomatosis cerebri include [[In vivo magnetic resonance spectroscopy|MR spectroscopy]], [[perfusion weighted imaging|MR perfusion]], [[PET scan]], and [[bone scan]].<ref name=aaa>Radiographic features of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref><ref name="pmid22740882">{{cite journal| author=Rajz GG, Nass D, Talianski E, Pfeffer R, Spiegelmann R, Cohen ZR| title=Presentation patterns and outcome of gliomatosis cerebri. | journal=Oncol Lett | year= 2012 | volume= 3 | issue= 1 | pages= 209-213 | pmid=22740882 | doi=10.3892/ol.2011.445 | pmc=PMC3362440 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22740882 }} </ref><ref name="pmid20334921">{{cite journal| author=Desclée P, Rommel D, Hernalsteen D, Godfraind C, de Coene B, Cosnard G| title=Gliomatosis cerebri, imaging findings of 12 cases. | journal=J Neuroradiol | year= 2010 | volume= 37 | issue= 3 | pages= 148-58 | pmid=20334921 | doi=10.1016/j.neurad.2009.12.001 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20334921 }} </ref><ref name="pmid24212625">{{cite journal| author=Beauchesne P| title=Extra-neural metastases of malignant gliomas: myth or reality? | journal=Cancers (Basel) | year= 2011 | volume= 3 | issue= 1 | pages= 461-77 | pmid=24212625 | doi=10.3390/cancers3010461 | pmc=PMC3756372 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24212625 }} </ref> [[Radiotherapy]] and [[chemotherapy]] are recommended among all patients who develop gliomatosis cerebri. [[Temozolomide]] and [[PCV regimen|PCV 3 combination chemotherapy]] are the preferred drugs for the treatment of high-grade and low-grade gliomatosis cerebri, respectively.<ref name="pmid21301914">{{cite journal| author=Inoue T, Kumabe T, Kanamori M, Sonoda Y, Watanabe M, Tominaga T| title=Prognostic factors for patients with gliomatosis cerebri: retrospective analysis of 17 consecutive cases. | journal=Neurosurg Rev | year= 2010 | volume= 34 | issue= 2 | pages= 197-208 | pmid=21301914 | doi=10.1007/s10143-010-0306-1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21301914 }} </ref><ref name="pmid11708542">{{cite journal| author=Hejazi N, Witzmann A, Hergan K| title=Gliomatosis cerebri: intra vitam stereotactic determination in two cases and review of the literature. | journal=Br J Neurosurg | year= 2001 | volume= 15 | issue= 5 | pages= 396-401 | pmid=11708542 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11708542 }} </ref><ref name="pmid15798516">{{cite journal| author=Sanson M, Napolitano M, Cartalat-Carel S, Taillibert S| title=[Gliomatosis cerebri]. | journal=Rev Neurol (Paris) | year= 2005 | volume= 161 | issue= 2 | pages= 173-81 | pmid=15798516 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15798516 }} </ref> Supportive therapy for gliomatosis cerebri includes [[anticonvulsants]] and [[corticosteroids]].<ref name="pmid22740882">{{cite journal| author=Rajz GG, Nass D, Talianski E, Pfeffer R, Spiegelmann R, Cohen ZR| title=Presentation patterns and outcome of gliomatosis cerebri. | journal=Oncol Lett | year= 2012 | volume= 3 | issue= 1 | pages= 209-213 | pmid=22740882 | doi=10.3892/ol.2011.445 | pmc=PMC3362440 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22740882 }} </ref> Surgery is not the first-line treatment option for patients with gliomatosis cerebri. | | Gliomatosis cerebri is a rare primary [[brain tumor]]. According to WHO, gliomatosis cerebri is classified as a distinct nosological entity among other glial tumors of the central nervous system. Gliomatosis cerebri is defined as a diffusely infiltrating neuroepithelial tumor, which involves at least three cerebral lobes and occasionally [[infratentorial]] structures or the [[spinal cord]]. The [[brain]] architecture is commonly preserved, with [[neurons]] being spared, and the mass effect is minimal. Gliomatosis cerebri was first reported by Landau in 1910. Since then, several physicians published isolated cases of gliomatosis cerebri and only later in 1938, it was completely studied and described by Nevin. 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, [[cyst|microcyst]]s, and [[Mitoses|mitotic figures]] without the presence of any [[necrosis]] or microvascular proliferation. Gliomatosis cerebri is a rare [[brain tumor]]. The incidence of gliomatosis cerebri is estimated to be less than 100 cases per year in the United States. Gliomatosis cerebri is a disease that tends to affect the middle-aged population. The peak incidence for gliomatosis cerebri is 20-40 years. The median age at diagnosis is 34 years.Males are more commonly affected with gliomatosis cerebri than females. The male to female ratio is approximately 1.5 to 1.If left untreated, patients with gliomatosis cerebri may progress to develop focal neurological deficits, [[altered mental status]], [[hydrocephalus]], [[brain herniation]], and ultimately death. Transformation into glioblastoma (grade 4) may occur a few years later. Common complications of gliomatosis cerebri include [[brain herniation]], [[hydrocephalus]], [[coma]], [[metastasis]], recurrence, [[benign intracranial hypertension]], and side effects of [[radiotherapy]] and [[chemotherapy]]. Prognosis of gliomatosis cerebri is generally poor, and the 5-year survival rate of patients with treatment is approximately 17.7%. Symptoms of gliomatosis cerebri include [[headache]], [[nausea]], [[vomiting]], [[seizure]], [[Gait ataxia|loss of balance]], [[memory loss]], [[personality changes]], [[Cognition|cognitive problems]], [[confusion]], [[diplopia]], [[Dysphagia|difficulty in swallowing]], [[Dysarthria|difficulty in speech]], [[Weakness|motor weakness]], and [[Numbness|facial numbness]].On head CT scan, gliomatosis cerebri is characterized by an isodense and hypoattenuated mass with ill-defined asymmetry and lack of mass effect. On brain MRI, gliomatosis cerebri is characterized by bilateral and diffuse infiltrative tumor which is isointense or hypointense on T1-weighted images and hyperintense on T2-weighted and FLAIR images. Other imaging studies for gliomatosis cerebri include [[In vivo magnetic resonance spectroscopy|MR spectroscopy]], [[perfusion weighted imaging|MR perfusion]], [[PET scan]], and [[bone scan]]. [[Radiotherapy]] and [[chemotherapy]] are recommended among all patients who develop gliomatosis cerebri. [[Temozolomide]] and [[PCV regimen|PCV 3 combination chemotherapy]] are the preferred drugs for the treatment of high-grade and low-grade gliomatosis cerebri, respectively. Supportive therapy for gliomatosis cerebri includes [[anticonvulsants]] and [[corticosteroids]]. Surgery is not the first-line treatment option for patients with gliomatosis cerebri. |
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| ==Historical Perspective== | | ==Historical Perspective== |
| Gliomatosis cerebri was first reported by Landau in 1910. Since then, several physicians published isolated cases of gliomatosis cerebri and only later in 1938, it was completely studied and described by Nevin.<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> | | Gliomatosis cerebri was first reported by Landau in 1910. Since then, several physicians published isolated cases of gliomatosis cerebri and only later in 1938, it was completely studied and described by Nevin. |
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| ==Classification== | | ==Classification== |
| Gliomatosis cerebri may be classified into several subtypes based on the origin (primary and secondary) and the type of tumor cell ([[astrocyte|astrocytic]], [[oligodendrocyte|oligodendroglial]], and mixed).<ref name=aaa>Classification of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref><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><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><ref name="pmid15277619">{{cite journal| author=Sanson M, Cartalat-Carel S, Taillibert S, Napolitano M, Djafari L, Cougnard J et al.| title=Initial chemotherapy in gliomatosis cerebri. | journal=Neurology | year= 2004 | volume= 63 | issue= 2 | pages= 270-5 | pmid=15277619 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15277619 }} </ref> | | Gliomatosis cerebri may be classified into several subtypes based on the origin (primary and secondary) and the type of tumor cell ([[astrocyte|astrocytic]], [[oligodendrocyte|oligodendroglial]], and mixed). |
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| ==Pathophysiology== | | ==Pathophysiology== |
| 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, [[cyst|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]]. Gliomatosis cerebri may be associated with [[neurofibromatosis type 1]] and [[pilomatricoma]]. 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, [[cyst|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]]. |
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| ==Causes== | | ==Causes== |
| Common causes of gliomatosis cerebri include [[mutation|genetic mutations]]. The genes associated with the etiology of gliomatosis cerebri include ''[[p53]]'',''[[OLIG2]]'', ''[[Ki-67 (Biology)|Ki-67]]'', ''[[EGFR]]'', ''[[PTEN]]'', ''[[VCAM1]]'', ''[[VEGF]]'', and [[gene|genes]] on [[chromosome 7|chromosome 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> | | Common causes of gliomatosis cerebri include [[mutation|genetic mutations]]. The genes associated with the etiology of gliomatosis cerebri include ''[[p53]]'',''[[OLIG2]]'', ''[[Ki-67 (Biology)|Ki-67]]'', ''[[EGFR]]'', ''[[PTEN]]'', ''[[VCAM1]]'', ''[[VEGF]]'', and [[gene|genes]] on [[chromosome 7|chromosome 7q]], [[chromosome 10|10q]], and [[chromosome 13|13q]]. |
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| ==Differentiating brain tumors from other diseases== | | ==Differentiating brain tumors from other diseases== |
| Gliomatosis cerebri must be differentiated from [[progressive multifocal leukoencephalopathy]], [[multiple sclerosis]], [[marburg virus|Marburg disease]], [[Glioblastoma|multicentric glioblastoma]], [[primary CNS lymphoma]], [[Encephalitis|viral encephalitis]], [[acute disseminated encephalomyelitis]], [[Vasculitis|CNS vasculitis]], [[Behçet's disease]], [[venous sinus thrombosis]], [[stroke]], [[Gerstmann syndrome]], [[Leptomeninges|leptomeningeal gliomatosis]], [[Alzheimer's disease]], [[Lewy body dementia]], and [[parkinsonism]].<ref name=bbb>Differential diagnosis of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref><ref name="pmid18284707">{{cite journal| author=Duron E, Lazareth A, Gaubert JY, Raso C, Hanon O, Rigaud AS| title=Gliomatosis cerebri presenting as rapidly progressive dementia and parkinsonism in an elderly woman: a case report. | journal=J Med Case Rep | year= 2008 | volume= 2 | issue= | pages= 53 | pmid=18284707 | doi=10.1186/1752-1947-2-53 | pmc=PMC2263063 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18284707 }} </ref><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><ref name="pmid20334921">{{cite journal| author=Desclée P, Rommel D, Hernalsteen D, Godfraind C, de Coene B, Cosnard G| title=Gliomatosis cerebri, imaging findings of 12 cases. | journal=J Neuroradiol | year= 2010 | volume= 37 | issue= 3 | pages= 148-58 | pmid=20334921 | doi=10.1016/j.neurad.2009.12.001 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20334921 }} </ref> | | Gliomatosis cerebri must be differentiated from [[progressive multifocal leukoencephalopathy]], [[multiple sclerosis]], [[marburg virus|Marburg disease]], [[Glioblastoma|multicentric glioblastoma]], [[primary CNS lymphoma]], [[Encephalitis|viral encephalitis]], [[acute disseminated encephalomyelitis]], [[Vasculitis|CNS vasculitis]], [[Behçet's disease]], [[venous sinus thrombosis]], [[stroke]], [[Gerstmann syndrome]], [[Leptomeninges|leptomeningeal gliomatosis]], [[Alzheimer's disease]], [[Lewy body dementia]], and [[parkinsonism]]. |
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| ==Epidemiology and Demographics== | | ==Epidemiology and Demographics== |
| Gliomatosis cerebri is a rare [[brain tumor]]. The incidence of gliomatosis cerebri is estimated to be less than 100 cases per year in the United States.<ref name=epi> Gliomatosis cerebri international registry. http://gcregistry.com/</ref> Gliomatosis cerebri is a disease that tends to affect the middle-aged population. The peak incidence for gliomatosis cerebri is 20-40 years.<ref name=aaa>Epidemiology of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref> The median age at diagnosis is 34 years.<ref name="pmid9810441">{{cite journal| author=Kim DG, Yang HJ, Park IA, Chi JG, Jung HW, Han DH et al.| title=Gliomatosis cerebri: clinical features, treatment, and prognosis. | journal=Acta Neurochir (Wien) | year= 1998 | volume= 140 | issue= 8 | pages= 755-62 | pmid=9810441 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9810441 }} </ref> Males are more commonly affected with gliomatosis cerebri than females. The male to female ratio is approximately 1.5 to 1.<ref name=aaa>Epidemiology of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref> | | Gliomatosis cerebri is a rare [[brain tumor]]. The incidence of gliomatosis cerebri is estimated to be less than 100 cases per year in the United States. Gliomatosis cerebri is a disease that tends to affect the middle-aged population. The peak incidence for gliomatosis cerebri is 20-40 years. The median age at diagnosis is 34 years. Males are more commonly affected with gliomatosis cerebri than females. The male to female ratio is approximately 1.5 to 1. |
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| ==Risk factors== | | ==Risk factors== |
| The most potent risk factor associated with the development of gliomatosis cerebri is [[neurofibromatosis type 1]].<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> | | The most potent risk factor associated with the development of gliomatosis cerebri is [[neurofibromatosis type 1]]. |
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| ==Screening== | | ==Screening== |
| There is insufficient evidence to recommend routine screening for gliomatosis cerebri.<ref name=screen>Early detection, diagnosis, and staging of brain tumors. American cancer society. http://www.cancer.org/cancer/braincnstumorsinadults/detailedguide/brain-and-spinal-cord-tumors-in-adults-detection</ref> | | There is insufficient evidence to recommend routine screening for gliomatosis cerebri. |
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| ==Natural History, Complications and Prognosis== | | ==Natural History, Complications and Prognosis== |
| If left untreated, patients with gliomatosis cerebri may progress to develop focal neurological deficits, [[altered mental status]], [[hydrocephalus]], [[brain herniation]], and ultimately death. Transformation into glioblastoma (grade 4) may occur a few years later.<ref name=aaa>Treatment and prognosis of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref> Common complications of gliomatosis cerebri include [[brain herniation]], [[hydrocephalus]], [[coma]], [[metastasis]], recurrence, [[benign intracranial hypertension]], and side effects of [[radiotherapy]] and [[chemotherapy]].<ref name="pmid12493121">{{cite journal| author=Weinberg JS, Rhines LD, Cohen ZR, Langford L, Levin VA| title=Posterior fossa decompression for life-threatening tonsillar herniation in patients with gliomatosis cerebri: report of three cases. | journal=Neurosurgery | year= 2003 | volume= 52 | issue= 1 | pages= 216-23; discussion 223 | pmid=12493121 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12493121 }} </ref><ref name=aaa>Treatment and prognosis of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref><ref name="pmidhttp://dx.doi.org/10.1017/S0012162201000214 (About">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=http://dx.doi.org/10.1017/S0012162201000214 (About | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10 }} </ref><ref name="pmid3124573">{{cite journal| author=Dickson DW, Horoupian DS, Thal LJ, Lantos G| title=Gliomatosis cerebri presenting with hydrocephalus and dementia. | journal=AJNR Am J Neuroradiol | year= 1988 | volume= 9 | issue= 1 | pages= 200-2 | pmid=3124573 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3124573 }} </ref><ref name="pmid16617385">{{cite journal| author=Krutsay M, Sipos G| title=[Gliomatosis cerebri]. | journal=Magy Onkol | year= 2006 | volume= 50 | issue= 1 | pages= 55-8 | pmid=16617385 | doi=HUON.2006.50.1.0055 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16617385 }} </ref><ref name="pmid10328549">{{cite journal| author=Kannuki S, Hirose T, Horiguchi H, Kageji T, Nagahiro S| title=Gliomatosis cerebri with secondary glioblastoma formation: report of two cases. | journal=Brain Tumor Pathol | year= 1998 | volume= 15 | issue= 2 | pages= 111-6 | pmid=10328549 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10328549 }} </ref> Prognosis of gliomatosis cerebri is generally poor, and the 5-year survival rate of patients with treatment is approximately 17.7%.<ref name="pmid21301914">{{cite journal| author=Inoue T, Kumabe T, Kanamori M, Sonoda Y, Watanabe M, Tominaga T| title=Prognostic factors for patients with gliomatosis cerebri: retrospective analysis of 17 consecutive cases. | journal=Neurosurg Rev | year= 2010 | volume= 34 | issue= 2 | pages= 197-208 | pmid=21301914 | doi=10.1007/s10143-010-0306-1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21301914 }} </ref><ref name="pmid1759182">{{cite journal| author=Ross IB, Robitaille Y, Villemure JG, Tampieri D| title=Diagnosis and management of gliomatosis cerebri: recent trends. | journal=Surg Neurol | year= 1991 | volume= 36 | issue= 6 | pages= 431-40 | pmid=1759182 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1759182 }} </ref> Median survival of patients who are treated with [[radiotherapy|whole brain radiotherapy]] alone is 13.7 months (range 1–35), whereas those who are treated with combined [[radiotherapy|whole brain radiation therapy]] and [[chemotherapy]] have a median survival of 26.14 months (range 6–42).<ref name="pmid22740882">{{cite journal| author=Rajz GG, Nass D, Talianski E, Pfeffer R, Spiegelmann R, Cohen ZR| title=Presentation patterns and outcome of gliomatosis cerebri. | journal=Oncol Lett | year= 2012 | volume= 3 | issue= 1 | pages= 209-213 | pmid=22740882 | doi=10.3892/ol.2011.445 | pmc=PMC3362440 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22740882 }} </ref> | | If left untreated, patients with gliomatosis cerebri may progress to develop focal neurological deficits, [[altered mental status]], [[hydrocephalus]], [[brain herniation]], and ultimately death. Transformation into glioblastoma (grade 4) may occur a few years later. Common complications of gliomatosis cerebri include [[brain herniation]], [[hydrocephalus]], [[coma]], [[metastasis]], recurrence, [[benign intracranial hypertension]], and side effects of [[radiotherapy]] and [[chemotherapy]]. Prognosis of gliomatosis cerebri is generally poor, and the 5-year survival rate of patients with treatment is approximately 17.7%. Median survival of patients who are treated with [[radiotherapy|whole brain radiotherapy]] alone is 13.7 months (range 1–35), whereas those who are treated with combined [[radiotherapy|whole brain radiation therapy]] and [[chemotherapy]] have a median survival of 26.14 months (range 6–42). |
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| ==Staging== | | ==Staging== |
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| ==History and Symptoms== | | ==History and Symptoms== |
| When evaluating a patient for gliomatosis cerebri, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history review. Other specific areas of focus when obtaining the history include review of common associated conditions such as [[neurofibromatosis type 1]].<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> Symptoms of gliomatosis cerebri include [[headache]], [[nausea]], [[vomiting]], [[seizure]], [[Gait ataxia|loss of balance]], [[memory loss]], [[personality changes]], [[Cognition|cognitive problems]], [[confusion]], [[diplopia]], [[Dysphagia|difficulty in swallowing]], [[Dysarthria|difficulty in speech]], [[Weakness|motor weakness]], and [[Numbness|facial numbness]].<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> | | When evaluating a patient for gliomatosis cerebri, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history review. Other specific areas of focus when obtaining the history include review of common associated conditions such as [[neurofibromatosis type 1]]. Symptoms of gliomatosis cerebri include [[headache]], [[nausea]], [[vomiting]], [[seizure]], [[Gait ataxia|loss of balance]], [[memory loss]], [[personality changes]], [[Cognition|cognitive problems]], [[confusion]], [[diplopia]], [[Dysphagia|difficulty in swallowing]], [[Dysarthria|difficulty in speech]], [[Weakness|motor weakness]], and [[Numbness|facial numbness]]. |
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| ==Physical examination== | | ==Physical examination== |
| Common physical examination findings of gliomatosis cerebri include [[dysphagia]], [[dysarthria]], [[nystagmus]], [[papilledema]], [[hemiparesis]], [[Paresthesia|facial paresthesia]], [[vision loss]], [[ataxia]], [[Dementia|mental status changes]], [[aphasia]], and focal neurological defects ([[Corticospinal tract|corticospinal tract defects]], [[Spinocerebellar tract|spinocerebellar tract defects]], and [[Nerve palsy|cranioneuropathies]]).<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="pmid22740882">{{cite journal| author=Rajz GG, Nass D, Talianski E, Pfeffer R, Spiegelmann R, Cohen ZR| title=Presentation patterns and outcome of gliomatosis cerebri. | journal=Oncol Lett | year= 2012 | volume= 3 | issue= 1 | pages= 209-213 | pmid=22740882 | doi=10.3892/ol.2011.445 | pmc=PMC3362440 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22740882 }} </ref> | | Common physical examination findings of gliomatosis cerebri include [[dysphagia]], [[dysarthria]], [[nystagmus]], [[papilledema]], [[hemiparesis]], [[Paresthesia|facial paresthesia]], [[vision loss]], [[ataxia]], [[Dementia|mental status changes]], [[aphasia]], and focal neurological defects ([[Corticospinal tract|corticospinal tract defects]], [[Spinocerebellar tract|spinocerebellar tract defects]], and [[Nerve palsy|cranioneuropathies]]). |
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| ==Laboratory Findings== | | ==Laboratory Findings== |
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| ==Chest X Ray== | | ==Chest X Ray== |
| Chest x-ray may be performed to detect [[metastases]] of gliomatosis cerebri to the [[lung]]s.<ref name="pmid24212625">{{cite journal| author=Beauchesne P| title=Extra-neural metastases of malignant gliomas: myth or reality? | journal=Cancers (Basel) | year= 2011 | volume= 3 | issue= 1 | pages= 461-77 | pmid=24212625 | doi=10.3390/cancers3010461 | pmc=PMC3756372 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24212625 }} </ref> | | Chest x-ray may be performed to detect [[metastases]] of gliomatosis cerebri to the [[lung]]s. |
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| ==CT== | | ==CT== |
| Head CT scan is helpful in the diagnosis of gliomatosis cerebri. On head CT scan, gliomatosis cerebri is characterized by an isodense and hypoattenuated mass with ill-defined asymmetry and lack of mass effect.<ref name=aaa>Radiographic features of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref> | | Head CT scan is helpful in the diagnosis of gliomatosis cerebri. On head CT scan, gliomatosis cerebri is characterized by an isodense and hypoattenuated mass with ill-defined asymmetry and lack of mass effect. |
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| ==MRI== | | ==MRI== |
| Brain MRI is helpful in the diagnosis of gliomatosis cerebri. On brain MRI, gliomatosis cerebri is characterized by bilateral and diffuse infiltrative tumor which is isointense or hypointense on T1-weighted images and hyperintense on T2-weighted and FLAIR images.<ref name=ddd>Radiographic features of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref><ref name="pmid9075253">{{cite journal| author=Onal C, Bayindir C, Siraneci R, Izgi N, Yalçin I, Altinel Z et al.| title=A serial CT scan and MRI verification of diffuse cerebrospinal gliomatosis: a case report with stereotactic diagnosis and radiological confirmation. | journal=Pediatr Neurosurg | year= 1996 | volume= 25 | issue= 2 | pages= 94-9 | pmid=9075253 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9075253 }} </ref> | | Brain MRI is helpful in the diagnosis of gliomatosis cerebri. On brain MRI, gliomatosis cerebri is characterized by bilateral and diffuse infiltrative tumor which is isointense or hypointense on T1-weighted images and hyperintense on T2-weighted and FLAIR images. |
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| ==Ultrasound== | | ==Ultrasound== |
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| ==Other Imaging Findings== | | ==Other Imaging Findings== |
| Other imaging studies for gliomatosis cerebri include [[In vivo magnetic resonance spectroscopy|MR spectroscopy]] (decreased [[N-Acetylaspartic acid|NAA]]/[[creatine]] ratio and elevated [[choline]]/[[creatine]] ratio, [[choline]]/[[N-Acetylaspartic acid|NAA]] ratio, and [[myo-inositol|myoinositol]]), [[perfusion weighted imaging|MR perfusion]] (low/normal relative cerebral blood flow), [[PET scan]] (markedly decreased accumulation of [[Florbetapir (18F)|[18F]-fluorodeoxyglucose]] on [[PET|F-18 FDG PET]], [[hypermetabolism]] on [[PET|C-11 methionine PET]], and marked increase in cerebral blood flow on [[PET|15(O)-water PET]]), and [[bone scan]] (metastasis to [[bones]]).<ref name=aaa>Radiographic features of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri</ref><ref name="pmid22740882">{{cite journal| author=Rajz GG, Nass D, Talianski E, Pfeffer R, Spiegelmann R, Cohen ZR| title=Presentation patterns and outcome of gliomatosis cerebri. | journal=Oncol Lett | year= 2012 | volume= 3 | issue= 1 | pages= 209-213 | pmid=22740882 | doi=10.3892/ol.2011.445 | pmc=PMC3362440 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22740882 }} </ref><ref name="pmid20334921">{{cite journal| author=Desclée P, Rommel D, Hernalsteen D, Godfraind C, de Coene B, Cosnard G| title=Gliomatosis cerebri, imaging findings of 12 cases. | journal=J Neuroradiol | year= 2010 | volume= 37 | issue= 3 | pages= 148-58 | pmid=20334921 | doi=10.1016/j.neurad.2009.12.001 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20334921 }} </ref><ref name="pmid24212625">{{cite journal| author=Beauchesne P| title=Extra-neural metastases of malignant gliomas: myth or reality? | journal=Cancers (Basel) | year= 2011 | volume= 3 | issue= 1 | pages= 461-77 | pmid=24212625 | doi=10.3390/cancers3010461 | pmc=PMC3756372 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24212625 }} </ref> | | Other imaging studies for gliomatosis cerebri include [[In vivo magnetic resonance spectroscopy|MR spectroscopy]] (decreased [[N-Acetylaspartic acid|NAA]]/[[creatine]] ratio and elevated [[choline]]/[[creatine]] ratio, [[choline]]/[[N-Acetylaspartic acid|NAA]] ratio, and [[myo-inositol|myoinositol]]), [[perfusion weighted imaging|MR perfusion]] (low/normal relative cerebral blood flow), [[PET scan]] (markedly decreased accumulation of [[Florbetapir (18F)|[18F]-fluorodeoxyglucose]] on [[PET|F-18 FDG PET]], [[hypermetabolism]] on [[PET|C-11 methionine PET]], and marked increase in cerebral blood flow on [[PET|15(O)-water PET]]), and [[bone scan]] (metastasis to [[bones]]). |
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| ==Other Diagnostic Studies== | | ==Other Diagnostic Studies== |
| Other diagnostic studies for gliomatosis cerebri include [[biopsy]], which demonstrates [[glial cells]] resembling [[astrocyte]]s, [[oligodendrocyte]]s, or undifferentiated cells with [[atypia]] and [[mitoses]].<ref name="pmid1759182">{{cite journal| author=Ross IB, Robitaille Y, Villemure JG, Tampieri D| title=Diagnosis and management of gliomatosis cerebri: recent trends. | journal=Surg Neurol | year= 1991 | volume= 36 | issue= 6 | pages= 431-40 | pmid=1759182 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1759182 }} </ref> | | Other diagnostic studies for gliomatosis cerebri include [[biopsy]], which demonstrates [[glial cells]] resembling [[astrocyte]]s, [[oligodendrocyte]]s, or undifferentiated cells with [[atypia]] and [[mitoses]]. |
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| ==Medical Therapy== | | ==Medical Therapy== |
| [[Radiotherapy]] and [[chemotherapy]] are recommended among all patients who develop gliomatosis cerebri. [[Temozolomide]] and [[PCV regimen|PCV 3 combination chemotherapy]] are the preferred drugs for the treatment of high-grade and low-grade gliomatosis cerebri, respectively.<ref name="pmid21301914">{{cite journal| author=Inoue T, Kumabe T, Kanamori M, Sonoda Y, Watanabe M, Tominaga T| title=Prognostic factors for patients with gliomatosis cerebri: retrospective analysis of 17 consecutive cases. | journal=Neurosurg Rev | year= 2010 | volume= 34 | issue= 2 | pages= 197-208 | pmid=21301914 | doi=10.1007/s10143-010-0306-1 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21301914 }} </ref><ref name="pmid11708542">{{cite journal| author=Hejazi N, Witzmann A, Hergan K| title=Gliomatosis cerebri: intra vitam stereotactic determination in two cases and review of the literature. | journal=Br J Neurosurg | year= 2001 | volume= 15 | issue= 5 | pages= 396-401 | pmid=11708542 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=11708542 }} </ref><ref name="pmid15798516">{{cite journal| author=Sanson M, Napolitano M, Cartalat-Carel S, Taillibert S| title=[Gliomatosis cerebri]. | journal=Rev Neurol (Paris) | year= 2005 | volume= 161 | issue= 2 | pages= 173-81 | pmid=15798516 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15798516 }} </ref> Supportive therapy for gliomatosis cerebri includes [[anticonvulsants]] and [[corticosteroids]].<ref name="pmid22740882">{{cite journal| author=Rajz GG, Nass D, Talianski E, Pfeffer R, Spiegelmann R, Cohen ZR| title=Presentation patterns and outcome of gliomatosis cerebri. | journal=Oncol Lett | year= 2012 | volume= 3 | issue= 1 | pages= 209-213 | pmid=22740882 | doi=10.3892/ol.2011.445 | pmc=PMC3362440 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22740882 }} </ref> | | [[Radiotherapy]] and [[chemotherapy]] are recommended among all patients who develop gliomatosis cerebri. [[Temozolomide]] and [[PCV regimen|PCV 3 combination chemotherapy]] are the preferred drugs for the treatment of high-grade and low-grade gliomatosis cerebri, respectively. Supportive therapy for gliomatosis cerebri includes [[anticonvulsants]] and [[corticosteroids]]. |
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| ==Surgery== | | ==Surgery== |
| Surgery is not the first-line treatment option for patients with gliomatosis cerebri. [[Hydrocephalus surgery|CSF shunting]] is usually reserved for patients with [[hydrocephalus]].<ref name=Hydro>Treatment and prognosis of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. http://radiopaedia.org/articles/gliomatosis-cerebri</ref> | | Surgery is not the first-line treatment option for patients with gliomatosis cerebri. [[Hydrocephalus surgery|CSF shunting]] is usually reserved for patients with [[hydrocephalus]]. |
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| ==Primary Prevention== | | ==Primary Prevention== |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]
Overview
Gliomatosis cerebri is a rare primary brain tumor. According to WHO, gliomatosis cerebri is classified as a distinct nosological entity among other glial tumors of the central nervous system. Gliomatosis cerebri is defined as a diffusely infiltrating neuroepithelial tumor, which involves at least three cerebral lobes and occasionally infratentorial structures or the spinal cord. The brain architecture is commonly preserved, with neurons being spared, and the mass effect is minimal. Gliomatosis cerebri was first reported by Landau in 1910. Since then, several physicians published isolated cases of gliomatosis cerebri and only later in 1938, it was completely studied and described by Nevin. 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 a rare brain tumor. The incidence of gliomatosis cerebri is estimated to be less than 100 cases per year in the United States. Gliomatosis cerebri is a disease that tends to affect the middle-aged population. The peak incidence for gliomatosis cerebri is 20-40 years. The median age at diagnosis is 34 years.Males are more commonly affected with gliomatosis cerebri than females. The male to female ratio is approximately 1.5 to 1.If left untreated, patients with gliomatosis cerebri may progress to develop focal neurological deficits, altered mental status, hydrocephalus, brain herniation, and ultimately death. Transformation into glioblastoma (grade 4) may occur a few years later. Common complications of gliomatosis cerebri include brain herniation, hydrocephalus, coma, metastasis, recurrence, benign intracranial hypertension, and side effects of radiotherapy and chemotherapy. Prognosis of gliomatosis cerebri is generally poor, and the 5-year survival rate of patients with treatment is approximately 17.7%. Symptoms of gliomatosis cerebri include headache, nausea, vomiting, seizure, loss of balance, memory loss, personality changes, cognitive problems, confusion, diplopia, difficulty in swallowing, difficulty in speech, motor weakness, and facial numbness.On head CT scan, gliomatosis cerebri is characterized by an isodense and hypoattenuated mass with ill-defined asymmetry and lack of mass effect. On brain MRI, gliomatosis cerebri is characterized by bilateral and diffuse infiltrative tumor which is isointense or hypointense on T1-weighted images and hyperintense on T2-weighted and FLAIR images. Other imaging studies for gliomatosis cerebri include MR spectroscopy, MR perfusion, PET scan, and bone scan. Radiotherapy and chemotherapy are recommended among all patients who develop gliomatosis cerebri. Temozolomide and PCV 3 combination chemotherapy are the preferred drugs for the treatment of high-grade and low-grade gliomatosis cerebri, respectively. Supportive therapy for gliomatosis cerebri includes anticonvulsants and corticosteroids. Surgery is not the first-line treatment option for patients with gliomatosis cerebri.
Historical Perspective
Gliomatosis cerebri was first reported by Landau in 1910. Since then, several physicians published isolated cases of gliomatosis cerebri and only later in 1938, it was completely studied and described by Nevin.
Classification
Gliomatosis cerebri may be classified into several subtypes based on the origin (primary and secondary) and the type of tumor cell (astrocytic, oligodendroglial, and mixed).
Pathophysiology
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. 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.
Causes
Common causes of gliomatosis cerebri include genetic mutations. The genes associated with the etiology of gliomatosis cerebri include p53,OLIG2, Ki-67, EGFR, PTEN, VCAM1, VEGF, and genes on chromosome 7q, 10q, and 13q.
Differentiating brain tumors from other diseases
Gliomatosis cerebri must be differentiated from progressive multifocal leukoencephalopathy, multiple sclerosis, Marburg disease, multicentric glioblastoma, primary CNS lymphoma, viral encephalitis, acute disseminated encephalomyelitis, CNS vasculitis, Behçet's disease, venous sinus thrombosis, stroke, Gerstmann syndrome, leptomeningeal gliomatosis, Alzheimer's disease, Lewy body dementia, and parkinsonism.
Epidemiology and Demographics
Gliomatosis cerebri is a rare brain tumor. The incidence of gliomatosis cerebri is estimated to be less than 100 cases per year in the United States. Gliomatosis cerebri is a disease that tends to affect the middle-aged population. The peak incidence for gliomatosis cerebri is 20-40 years. The median age at diagnosis is 34 years. Males are more commonly affected with gliomatosis cerebri than females. The male to female ratio is approximately 1.5 to 1.
Risk factors
The most potent risk factor associated with the development of gliomatosis cerebri is neurofibromatosis type 1.
Screening
There is insufficient evidence to recommend routine screening for gliomatosis cerebri.
Natural History, Complications and Prognosis
If left untreated, patients with gliomatosis cerebri may progress to develop focal neurological deficits, altered mental status, hydrocephalus, brain herniation, and ultimately death. Transformation into glioblastoma (grade 4) may occur a few years later. Common complications of gliomatosis cerebri include brain herniation, hydrocephalus, coma, metastasis, recurrence, benign intracranial hypertension, and side effects of radiotherapy and chemotherapy. Prognosis of gliomatosis cerebri is generally poor, and the 5-year survival rate of patients with treatment is approximately 17.7%. Median survival of patients who are treated with whole brain radiotherapy alone is 13.7 months (range 1–35), whereas those who are treated with combined whole brain radiation therapy and chemotherapy have a median survival of 26.14 months (range 6–42).
Staging
There is no established system for the staging of gliomatosis cerebri.
History and Symptoms
When evaluating a patient for gliomatosis cerebri, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough family and past medical history review. Other specific areas of focus when obtaining the history include review of common associated conditions such as neurofibromatosis type 1. Symptoms of gliomatosis cerebri include headache, nausea, vomiting, seizure, loss of balance, memory loss, personality changes, cognitive problems, confusion, diplopia, difficulty in swallowing, difficulty in speech, motor weakness, and facial numbness.
Physical examination
Common physical examination findings of gliomatosis cerebri include dysphagia, dysarthria, nystagmus, papilledema, hemiparesis, facial paresthesia, vision loss, ataxia, mental status changes, aphasia, and focal neurological defects (corticospinal tract defects, spinocerebellar tract defects, and cranioneuropathies).
Laboratory Findings
There are no diagnostic lab findings associated with gliomatosis cerebri.
Chest X Ray
Chest x-ray may be performed to detect metastases of gliomatosis cerebri to the lungs.
CT
Head CT scan is helpful in the diagnosis of gliomatosis cerebri. On head CT scan, gliomatosis cerebri is characterized by an isodense and hypoattenuated mass with ill-defined asymmetry and lack of mass effect.
MRI
Brain MRI is helpful in the diagnosis of gliomatosis cerebri. On brain MRI, gliomatosis cerebri is characterized by bilateral and diffuse infiltrative tumor which is isointense or hypointense on T1-weighted images and hyperintense on T2-weighted and FLAIR images.
Ultrasound
There are no ultrasound findings associated with gliomatosis cerebri.
Other Imaging Findings
Other imaging studies for gliomatosis cerebri include MR spectroscopy (decreased NAA/creatine ratio and elevated choline/creatine ratio, choline/NAA ratio, and myoinositol), MR perfusion (low/normal relative cerebral blood flow), PET scan (markedly decreased accumulation of [18F]-fluorodeoxyglucose on F-18 FDG PET, hypermetabolism on C-11 methionine PET, and marked increase in cerebral blood flow on 15(O)-water PET), and bone scan (metastasis to bones).
Other Diagnostic Studies
Other diagnostic studies for gliomatosis cerebri include biopsy, which demonstrates glial cells resembling astrocytes, oligodendrocytes, or undifferentiated cells with atypia and mitoses.
Medical Therapy
Radiotherapy and chemotherapy are recommended among all patients who develop gliomatosis cerebri. Temozolomide and PCV 3 combination chemotherapy are the preferred drugs for the treatment of high-grade and low-grade gliomatosis cerebri, respectively. Supportive therapy for gliomatosis cerebri includes anticonvulsants and corticosteroids.
Surgery
Surgery is not the first-line treatment option for patients with gliomatosis cerebri. CSF shunting is usually reserved for patients with hydrocephalus.
Primary Prevention
There is no established method for prevention of gliomatosis cerebri.
Secondary Prevention
There are no secondary preventive measures available for gliomatosis cerebri.
References
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