Gliomatosis cerebri: post-mortem molecular and immunohistochemical analyses in a case treated with thalidomide.
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 with a bioptically diagnosed fibrillary astrocytoma. The administration of thalidomide, which was suggested to be beneficial in the treatment of human cancers, had no substantial clinical effect on our patient. Autopsy studies revealed a diffuse infiltration of the frontal and temporal lobes of the right hemisphere, brainstem, and the leptomeninges covering the whole spinal cord by an astrocytic tumor, which showed features both of low-grade astrocytoma and glioblastoma multiforme. No mutations in the p53 and PTEN tumor suppressor genes were found; immunoreactivities for p53, PTEN, and EGFR could not be detected.
Mawrin
C
C
Department of Neuropathology, Otto-von-Guericke University, Magdeburg, Germany. christian.mawrin@medizin.uni-magdeburg.de
Aumann
V
V
Kirches
E
E
Schneider-Stock
R
R
Scherlach
C
C
Vogel
S
S
Mittler
U
U
Dietzmann
K
K
Krause
G
G
Weis
S
S
D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D001932:Brain Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D003937:Diagnosis, Differential; D066246:ErbB Receptors / Q000235:genetics / Q000378:metabolism; D017809:Fatal Outcome; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018302:Neoplasms, Neuroepithelial / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D051059:PTEN Phosphohydrolase; D010744:Phosphoric Monoester Hydrolases / Q000235:genetics / Q000378:metabolism; D013125:Spinal Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D013792:Thalidomide / Q000627:therapeutic use*; D016159:Tumor Suppressor Protein p53 / Q000235:genetics / Q000378:metabolism; D025521:Tumor Suppressor Proteins / Q000235:genetics / Q000378:metabolism
D016159:Tumor Suppressor Protein p53; D025521:Tumor Suppressor Proteins; D013792:Thalidomide; D066246:ErbB Receptors; D010744:Phosphoric Monoester Hydrolases; D051059:PTEN Phosphohydrolase; C494929:PTEN protein, human