A major development in progress towards slowing or stopping the development of dermal neurofibromas has been discovered by Dr. Lu Le and his team at University of Texas Southwestern. This research is being done as part of The Giorgio Foundations’s Dermal Neurofibroma Consortium.
Currently, the only treatment for neurofibromas is surgical removal of the most uncomfortable and most disfiguring of the skin tumors. It would be impossible to remove them all.
“For the first time we have a mouse model that develops different types of neurofibromas inside the body and on the skin, just like in humans. Because of this model, we now know the exact origin of these two types of tumors. If you know where the tumor begins, and you know the end result, then you can follow the steps in the occurrence of the tumor and figure out how to interrupt the development of the tumors,” said Dr. Le, who treats NF1 patients as well as does research on the condition.
The researchers found that the protein Hox-B7 is a marker for the cell of origin for NF1 tumors. “It’s like a GPS system in a car. By making the Hox-B7 cells light up, we can follow the development of the tumor. It’s like branding,” said Dr. Le, the senior author of the study and a member of the Harold C. Simmons Comprehensive Cancer Center.
Another key discovery is that a parallel pathway, the Hippo pathway, can modify growth and development of these tumors. This is particularly important because treatments are being developed to block the Hippo pathway. “If you can control the Hippo pathway, you should be able to slow the development of neurofibromas, specifically in NF1 patients who also have genetic changes in their Hippo pathway,” Dr. Le said.
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