What is LFS?
Li-Fraumeni Syndrome (LFS) is an inherited familial predisposition to a wide range of certain, often rare, cancers.
This is due to a change (mutation) in a tumour suppressor gene known as TP53. The resulting p53 protein produced by the gene is damaged (or otherwise rendered malfunctioning), and is unable to help prevent malignant tumours from developing. Children and young adults are susceptible to developing several multiple cancers, most notably soft-tissue and bone sarcomas, breast cancer, brain tumours, adrenocortical carcinoma and acute leukaemia.
Other cancers seen in LFS patients include gastrointestinal cancers and cancers of the lung, kidney, thyroid, and skin, as well as in gonadal organs (ovarian, testicular, and prostate). It is important to note that not everyone with a TP53 gene mutation will necessarily develop cancer, but the risks are substantially higher than in the general population. A diagnosis of LFS is critically important so that affected families can seek appropriate genetic counselling as well as screening for early detection of cancer.
Brain tumours are quite complex - at present, over 130 different types of 'high grade' (cancerous) or 'low grade' (non-cancerous) brain tumours are known.
Brain tumours kill more children and adults under the age of 40 than any other cancer yet up until recently just 1% of the national spend on cancer research has been allocated to this devastating disease.
Find key information about brain tumours in adults and children including symptoms, diagnosis and treatments, and read advice on living with, or caring for someone with, a brain tumour.
For more details, visit: https://www.thebraintumourcharity.org/understanding-brain-tumours/