Giant cell glioblastoma

Giant cell glioblastoma is a variant of glioblastoma (along with epithelioid glioblastoma and giant cell glioblastoma) recognised in the current (2016) WHO classification of CNS tumours 7. This tumour was previously called monstrocellular tumour due to the macro size of its cells.

This neoplasm represents ~5% of GBM cases 1, with wider age range with a tendency to affect younger individuals than does conventional GBM 3, and accounts for about 1% of primary brain tumours.

The clinical features are similar to glioblastoma in general and patients could present a focal neurological deficit, symptoms of increased ICP, and seizures.

Giant cell glioblastomas are usually encountered in the cerebral hemispheres but can occur at any site in the central nervous system. There are some reports of extradural 6 and spinal leptomeningeal 5 metastases. 

As is the case with some gliosarcomas, giant cell glioblastomas often have abundant connective tissue resulting in firm and circumscribed tumours reminiscent of cerebral metastases or even meningiomas 7. In other instances, connective tissue is minimal and appearances are indistinguishable from glioblastomas

Giant cell glioblastoma is defined as a glioblastoma with a marked predominance of bizarre multinucleated giant cells that can be up to 0.5mm in diameter 1,7.

This tumour contains a high frequency of TP53 mutations (70-90%) and less commonly PTEN mutations (33%) and TERT mutations (25%) 2,7. EGFR amplification and IDH mutations are rare (i.e. they are IDH wild-type) 7

Giant cell glioblastomas has no distinguishing features when compared to the glioblastoma 4.

Giant cell glioblastoma overall survival is superior when compared to patients with conventional IDH wild-type glioblastoma, although it remains poor with only ~10% 5-year survival and a median survival of 11 to 13 months 1,3,7.

This tumour was first described by Alexander Schmincke (1877-1953), a German pathologist 1.

On imaging the differential is primarily: 

Histologically, pleomorphic xanthoastrocytoma (PXA) is one important differential diagnosis, as both have in common the presence of giant tumour cells, infiltration of lymphocytes, deposition of reticulin and gross circumscription 4. Neuronal markers, positive in PXAs, are negative in giant cell glioblastomas. 

Astrocytic tumour
Share article

Article information

rID: 29935
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Monstrocellular tumor
  • Monstrocellular tumour

Support Radiopaedia and see fewer ads

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.