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.
Treatment and prognosis
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.
History and etymology
This tumour was first described by Alexander Schmincke (1877-1953), a German pathologist 1.
On imaging the differential is primarily:
- conventional glioblastoma: usually indistinguishable
- cerebral metastases
- meningioma: if abutting the dura
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.
- 1. Kozak KR, Moody JS. Giant cell glioblastoma: a glioblastoma subtype with distinct epidemiology and superior prognosis. Neuro-oncology. 2009;11 (6): 833-41. doi:10.1215/15228517-2008-123 - Free text at pubmed - Pubmed citation
- 2. Peraud A, Watanabe K, Schwechheimer K et-al. Genetic profile of the giant cell glioblastoma. Lab. Invest. 1999;79 (2): 123-9. Pubmed citation
- 3. Margetts JC, Kalyan-Raman UP. Giant-celled glioblastoma of brain. A clinico-pathological and radiological study of ten cases (including immunohistochemistry and ultrastructure). Cancer. 1989;63 (3): 524-31. Pubmed citation
- 4. Valle-Folgueral JM, Mascarenhas L, Costa JA et-al. Giant cell glioblastoma: review of the literature and illustrated case. Neurocirugia (Astur). 2008;19 (4): 343-9. Pubmed citation
- 5. Chang CC, Kuwana N, Ito S et-al. Spinal leptomeningeal metastases of giant cell glioblastoma associated with subarachnoid haemorrhage: case report. J Clin Neurosci. 2001;8 (1): 56-9. doi:10.1054/jocn.2000.0745 - Pubmed citation
- 6. Shinmura F, Chen M, Itoh T et-al. An autopsy case of extraneural metastases of giant cell glioblastoma with intracerebral hemorrhage. No Shinkei Geka. 1986;13 (11): 1245-50. Pubmed citation
- 7. Louis DN, Ohgaki H, Wiestler OD et-al. The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol. 2007;114 (2): 97-109. Acta Neuropathol. (full text) - doi:10.1007/s00401-007-0243-4 - Free text at pubmed - Pubmed citation
- WHO classification of CNS tumours
- WHO grading of CNS tumours
- VASARI MRI feature set
- diffuse astrocytoma grading
- grade I:
- grade II:
- grade III
- grade IV:
- glioblastoma vs cerebral metastasis
- radiation-induced gliomas
- gliomatosis cerebri (growth pattern)
- specific locations
- treatment response
- Stupp protocol
- glioma treatment response assessment in clinical trials
- multicentric glioblastoma
- multifocal glioblastoma
- prognostic genetic markers