9381/3 Gliomatosis cerebri


Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72
ICD10: C71

/span> diffuse glial tumor which infiltrates the brain extensively, involving three or more lobes with diffuse enlargement of anatomic structures. It is frequently bilateral and often extends to infratentorial structures, even to the spinal cord. It is considered of astrocytic origin, although GFAP expression may be scant or absent. IDH1/2 mutations were not detected in a series of 11 classical cases of gliomastosis cerebri, indicating that they are derived from precursor cells different of those of diffuse astrocytomas 1
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Seiz M, Tuettenberg J, Meyer J, Essig M, Schmieder K, Mawrin C, von Deimling A, Hartmann C (2010)
Detection of IDH1 mutations in gliomatosis cerebri, but only in tumors with additional solid component: evidence for molecular subtypes.
Acta Neuropathol 120: 261-7

Occasionally, oligodendroglioma, including examples that exhibit codeletion of chromosomal arms 1p and 19q, and mixed oligoastrocytoma can also show the gliomatosis cerebri growth pattern.
Kleihues P, Cavenee WK (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Nervous System.
3rd Edition
IARC Press: Lyon 2000