Inside pLGG
Identifying the tumor’s biology
Ayiden, lives with pLGG.
Lives for basketball.
Understanding tumor biology may help with choosing the best available treatment
Before diving into how these pLGG tumors behave, it is important to first understand how "normal" cells function in the human body.
Normal cells need proteins to work properly in order to regulate growth.
These proteins work together and exchange information to control cell growth.
But, in pLGG, there may be a change in one of these proteins, resulting in continuous signaling and uncontrolled cell growth.
Learn more about pLGG biology and cell growth in the video below
The MAPK pathway
Every cell has signals that tell it when to grow and when to stop. In pLGG, a signal called the Mitogen-Activated Protein Kinase pathway, aka the MAPK pathway, can get stuck “on,” causing cells to keep growing when they shouldn’t. The MAPK pathway is a key driver in the development of pLGG.
Common gene changes in pLGG
Gene changes can lead to multiple cell function changes, including how cells grow.
BRAF = a gene that, when altered, may be a driver of pLGG growth is the gene most commonly altered in pLGG
Up to 75% of children with pLGG have some type of BRAF change
What is the BRAF gene?
BRAF is a gene that makes a protein that helps cells behave properly. BRAF gene changes have been found in many types of cancer, which may increase growth and the spread of cancer cells.
2 common changes in the BRAF gene include:
BRAF point mutation = 1 of the 2 most common types of BRAF alterations in pLGG (eg, BRAF V600E)—A specific change in the BRAF = a gene that, when altered, may be a driver of pLGG growth gene. The most common point mutation is the V600E mutation
BRAF fusion = 1 of the 2 most common types of BRAF alterations in pLGG (eg, KIAA1549-BRAF fusion)—When the BRAF = a gene that, when altered, may be a driver of pLGG growth gene joins another gene
While BRAF = a gene that, when altered, may be a driver of pLGG growth gene changes are the most common in pLGG tumors, other genes changes like FGFR1, NF1, NTRK, or other rarer mutations can occur in the MAPK pathway and increase the risk for developing pLGG tumors.
It is important to know if your child's pLGG has one of these BRAF = a gene that, when altered, may be a driver of pLGG growth Alteration = a type of genomic change (mutation or fusion) in the BRAF gene that may contribute to cancer development so that their care team can identify an appropriate treatment.
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