Gliomatosis cerebri natural history, complications, and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

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). Median survival without any form of treatment is reported raging from 7 to 18.5 months.

Natural History

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.^[1]^[2]

Complication

Common complications of gliomatosis cerebri include:^[3]^[1]^[4]^[2]

Prognosis

Prognosis of gliomatosis cerebri is generally poor, and the 5-year survival rate of patients with treatment is approximately 17.7%.^[5]^[6]

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).^[7] Median survival without any form of treatment is reported raging from 7 to 18.5 months. ^[8]

The prognosis of gliomatosis cerebri depends on the following:^[9]^[10]

Resectibility of the tumor mass
Age of the patient
- Age < 10 year is associated with worse prognosis.
Gender of the patient
- Males have better prognosis than females.
Predominant type of cell
- Oligodendroglial type of gliomatosis cerebri have a better prognosis.
Location of tumor
- Infratentorial tumor is associated with shorter survival.
Size of the tumor
Stage of the cancer
Primary diagnosis vs. recurrence

References

↑ ^1.0 ^1.1 Treatment and prognosis of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri
↑ ^2.0 ^2.1 Kannuki S, Hirose T, Horiguchi H, Kageji T, Nagahiro S (1998). “Gliomatosis cerebri with secondary glioblastoma formation: report of two cases”. Brain Tumor Pathol. 15 (2): 111–6. PMID 10328549.
↑ Weinberg JS, Rhines LD, Cohen ZR, Langford L, Levin VA (2003). “Posterior fossa decompression for life-threatening tonsillar herniation in patients with gliomatosis cerebri: report of three cases”. Neurosurgery. 52 (1): 216–23, discussion 223. PMID 12493121.
↑ Krutsay M, Sipos G (2006). “[Gliomatosis cerebri]”. Magy Onkol. 50 (1): 55–8. doi:HUON.2006.50.1.0055 Check |doi= value (help). PMID 16617385.
↑ Inoue T, Kumabe T, Kanamori M, Sonoda Y, Watanabe M, Tominaga T (2010). “Prognostic factors for patients with gliomatosis cerebri: retrospective analysis of 17 consecutive cases”. Neurosurg Rev. 34 (2): 197–208. doi:10.1007/s10143-010-0306-1. PMID 21301914.
↑ Ross IB, Robitaille Y, Villemure JG, Tampieri D (1991). “Diagnosis and management of gliomatosis cerebri: recent trends”. Surg Neurol. 36 (6): 431–40. PMID 1759182.
↑ Rajz GG, Nass D, Talianski E, Pfeffer R, Spiegelmann R, Cohen ZR (2012). “Presentation patterns and outcome of gliomatosis cerebri”. Oncol Lett. 3 (1): 209–213. doi:10.3892/ol.2011.445. PMC 3362440. PMID 22740882.
↑ “Gliomatosis cerebri: a consensus summary report from the Second International Gliomatosis cerebri Group Meeting, June 22–23, 2017, Bethesda, USA”.
↑ 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.
↑ 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.

Template:WikiDoc Sources

[aaa-1] 1.0 ^1.1 Treatment and prognosis of gliomatosis cerebri. Dr Henry Knipe and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/gliomatosis-cerebri

[pmid10328549-2] 2.0 ^2.1 Kannuki S, Hirose T, Horiguchi H, Kageji T, Nagahiro S (1998). “Gliomatosis cerebri with secondary glioblastoma formation: report of two cases”. Brain Tumor Pathol. 15 (2): 111–6. PMID 10328549.

[pmid12493121-3] Weinberg JS, Rhines LD, Cohen ZR, Langford L, Levin VA (2003). “Posterior fossa decompression for life-threatening tonsillar herniation in patients with gliomatosis cerebri: report of three cases”. Neurosurgery. 52 (1): 216–23, discussion 223. PMID 12493121.

[pmid16617385-4] Krutsay M, Sipos G (2006). “[Gliomatosis cerebri]”. Magy Onkol. 50 (1): 55–8. doi:HUON.2006.50.1.0055 Check |doi= value (help). PMID 16617385.

[pmid21301914-5] Inoue T, Kumabe T, Kanamori M, Sonoda Y, Watanabe M, Tominaga T (2010). “Prognostic factors for patients with gliomatosis cerebri: retrospective analysis of 17 consecutive cases”. Neurosurg Rev. 34 (2): 197–208. doi:10.1007/s10143-010-0306-1. PMID 21301914.

[pmid1759182-6] Ross IB, Robitaille Y, Villemure JG, Tampieri D (1991). “Diagnosis and management of gliomatosis cerebri: recent trends”. Surg Neurol. 36 (6): 431–40. PMID 1759182.

[pmid22740882-7] Rajz GG, Nass D, Talianski E, Pfeffer R, Spiegelmann R, Cohen ZR (2012). “Presentation patterns and outcome of gliomatosis cerebri”. Oncol Lett. 3 (1): 209–213. doi:10.3892/ol.2011.445. PMC 3362440. PMID 22740882.

[8] “Gliomatosis cerebri: a consensus summary report from the Second International Gliomatosis cerebri Group Meeting, June 22–23, 2017, Bethesda, USA”.

[pmid12325066-9] 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.

[pmid21339680-10] 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.

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