C. Mawrin et al., Gliomatosis cerebri: Post-mortem molecular and immunohistochemical analyses in a case treated with thalidomide, J NEURO-ONC, 55(1), 2001, pp. 11-17
Gliomatosis cerebri (GC) is a rare tumor of the central nervous system (CNS
) characterized by widespread diffuse infiltration of the brain and spinal
cord by neoplastic glial cells. We report the case of a 17-year-old boy wit
h a bioptically diagnosed fibrillary astrocytoma. The administration of tha
lidomide, which was suggested to be beneficial in the treatment of human ca
ncers, had no substantial clinical effect on our patient. Autopsy studies r
evealed a diffuse infiltration of the frontal and temporal lobes of the rig
ht hemisphere, brainstem, and the leptomeninges covering the whole spinal c
ord by an astrocytic tumor, which showed features both of low-grade astrocy
toma and glioblastoma multiforme. No mutations in the p53 and PTEN tumor su
ppressor genes were found; immunoreactivities for p53, PTEN, and EGFR could
not be detected.