Abstract
Gliomatosis cerebri was deleted as a tumor entity. Instead, it was defined to be a growth pattern under the topics “diffuse astrocytoma, IDH-wild-type,” “anaplastic astrocytoma, IDH-wild-type,” and “glioblastoma, IDH-wild-type.” The gliomatosis cerebri pattern shows extensive involvement of the CNS, with the affected area ranging from most of one cerebral hemisphere (three lobes or more) to both cerebral hemispheres with additional involvement of the deep gray matter structures, brain stem, cerebellum, and spinal cord.
Gliomatosis cerebri might show areas of tumor manifestation corresponding to diffuse astrocytoma (WHO grade II), anaplastic astrocytoma (WHO grade III), and glioblastoma (WHO grade IV).
Gliomatosis cerebri is thought to be a subtype of glioma with unusual infiltrative capacity or result from simultaneous neoplastic transformation at different regional sites (“field cancerization”). Monoclonal origin of gliomatosis cerebri with widespread tumor infiltration is considered.
Treatment consists in performing a biopsy for establishing the diagnosis as resection is usually impossible because of diffuse spread, followed by radiation and chemotherapy with temozolomide and PCV (procarbazine, carmustine, vincristine). Median survival ranges between 11 and 38 months and depends on age at onset (<42 years), Karnofsky performance status at time of clinical presentation (>80), WHO grade of the tumor, histologic subtype, Ki67, and MGMT promoter methylation status.
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Weis, S. et al. (2019). Gliomatosis Cerebri. In: Imaging Brain Diseases. Springer, Vienna. https://doi.org/10.1007/978-3-7091-1544-2_56
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DOI: https://doi.org/10.1007/978-3-7091-1544-2_56
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