Understanding inherited genetic risk of adult glioma – a review

open access

 ..... In this paper, we review current knowledge about inherited risk for primary adult glioma, specifically, glioblastoma, and grade II/III astrocytoma, oligodendroglioma, and oligoastrocytoma. We also discuss how inherited risk varies by histology and molecular subtypes characterized by acquired mutations. An information sheet is appended to help patients and their families understand these important concepts.....That several rare hereditary cancer syndromes greatly increase risk of glioma has been known for many years. These familial syndromes include neurofibromatosis, tuberous sclerosis, Lynch syndrome, Li-Fraumeni syndrome, melanoma-neural system tumor syndrome, and Ollier disease (Table 1).¹⁴

 It is very important to understand that inherited genetic risk variants, whether common or rare, with low or high penetrance, are neither necessary nor sufficient for glioma formation – rather, they contribute to risk. Even for people with familial syndromes, additional acquired mutations are required for tumorigenesis. Moreover, not everyone who inherits the same mutation necessarily develops cancer or the same type of cancer.

 Understanding inherited genetic risk of adult glioma – a review

Supplementary Data

Supplementary Data

• Supplementary Data - docx file
• Supplementary Table 1 - docx file
• Supplementary Table 2 - docx file

Table 1. Rare hereditary cancer syndromes that increase risk of glioma