Epithelioid glioblastoma

Last revised by Frank Gaillard on 21 Feb 2022

Epithelioid glioblastoma is a classic variant of glioblastoma (along with gliosarcoma and giant cell glioblastoma) recognized in the current (2021) WHO classification of CNS tumors as a subtype 4

Whether or not epithelioid glioblastomas are distinct from rhabdoid glioblastomas is at present unclear, however, the latter term should be avoided and is not recognized in the current WHO classification 2,3

It is important to note that true epithelial differentiation of glioblastomas (typically squamous) is very rarely seen in adults but is distinct from epithelioid glioblastoma 2

Unlike run-of-the-mill glioblastomas that are usually encountered in older adults, epithelioid glioblastomas have a predilection for young adults and children 1,3

Epithelioid glioblastomas are WHO grade 4 tumors 3. Interestingly, they sometimes co-exist with pleomorphic xanthoastrocytomas, however, their exact relationship is at present unclear 3

These tumors are heterogeneous with large epithelioid cells that have abundant eosinophilic cytoplasm, vesicular chromatin, and prominent nucleoli. These cells are reminiscent of melanoma cells. Rhabdoid cells are also sometimes encountered 1.

Immunohistochemistry demonstrates a mixture of astrocytic and epithelial markers 3

Approximately 50% of cases of epithelioid glioblastoma have BRAF V600E mutations 1-3

Epithelioid glioblastomas most frequently present as diencephalic or less frequently superficial cerebral hemispheric masses 1,2. Hemorrhage and leptomeningeal seeding are probably fairly common at the time of diagnosis 2

These tumors are indistinguishable from glioblastoma on imaging, with enhancement, necrosis and diffusion restriction present in the vast majority of tumors 2

Epithelioid glioblastomas are aggressive tumors with a poor prognosis, even worse than ordinary glioblastomas 3. In one series the median survival was only 169 days 2

These tumors also sometimes demonstrate systemic metastases, a very uncommon occurrence for other primary brain tumors 2

In children, the main differential is atypical teratoid/rhabdoid tumor (AT/RT), distinguished by universal lack of INI1 expression in AT/RT 2

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