|Contributor: James G. Smirniotopoulos, M.D. - Uniformed Services University|
|Peer Review/Approval: James G. Smirniotopoulos, M.D. - Uniformed Services University|
|ACR Codes: 1.3|
Gliomatosis Cerebri (GC) is a controversial designation for a diffusely infiltrating process affecting multiple areas of the CNS, yet without causing a focal mass. By some definitions, 2 or more cerebral lobes must be affected. Less than 200 cases are documented in the NLM (National Library of Medicine) article database.
One of the accepted hallmarks is increased cellularity without destruction of the infiltrated parenchyma. At one time considered to be a hamartomatous disorder, it is now generally accepted to be neoplastic. A retrospective review of 160 published cases showed a 50% mortality at 12 months from diagnosis.
There is considerable overlap of gliomatosis cerebri and diffuse low-grade astrocytoma. Some have questioned that making such a distinction is not only difficult, but perhaps unimportant. Others have suggested that the molecular biology of GC does not clearly demonstrate an origin from astrocytes.
Because of the non-destructive infiltration, patients usually present without any localizing signs and may have general dementia, loss of corticospinal tract function diffusely, siezures, and headache.
Reported cases with transformation and/or association of GC with focal areas of clear-cut anaplastic astroctoma or glioblastoma reinforce the concept of a relationship with diffuse astrocytoma.
"Gliomatosis cerebri is a rare, diffusely growing neuroepithelial tumor characterized by extensive brain infiltration involving more than two cerebral lobes. Among 13 patients with gliomatosis cerebri (median age, 46 years), biopsies showed features of diffuse astrocytoma (n = 4), oligoastrocytoma (n = 1), anaplastic astrocytoma (n = 5), anaplastic oligoastrocytoma (n = 1), or glioblastoma (n = 2). Molecular genetic investigation showed TP53 mutations in three of seven tumors and both PTEN mutation and epidermal growth factor receptor overexpression in one tumor. Amplification of CDK4 or MDM2 or homozygous deletion of CDKN2A was not detected. Three of 10 patients receiving radiotherapy showed a partial response (one patient) or had stable disease (two patients)
lasting for more than 1 year. Four of six patients treated with procarbazine, carmustine, vincristine chemotherapy demonstrated partial remission (one patient), minor response (two patients), or stable disease (one patient). Median survival time from diagnosis was 14 months (range, 4-91+ months). Infratentorial involvement was associated with shorter survival. We conclude that (1) the molecular
genetic alterations in gliomatosis cerebri resemble those in diffuse astrocytomas; (2) the prognosis of gliomatosis cerebri is variable but for at least 50% of patients as poor as for glioblastoma; and (3) some patients respond to radiotherapy and/or procarbazine, carmustine, vincristine chemotherapy."
Ann Neurol 2002 Oct;52(4):390-9
PMID: 12325066 [PubMed - indexed for MEDLINE]
Editorial by Peter C. Burger, M.D. Ann Neurol 2002;52:389
"In the meantime, for all of its radiological and clinical appeal, gliomatosis cerebri is not a specific entity."
1) Herrlinger U, Felsberg J, Kuker W, Bornemann A, Plasswilm L, Knobbe CB, Strik H, Wick W, Meyermann R, Dichgans J, Bamberg M, Reifenberger G, Weller M: Gliomatosis cerebri: molecular pathology and clinical course.
Ann Neurol 2002 Oct;52(4):390-9
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