Meet Dr. Angela Hirbe, the 2024 Project Dream Recipient. She is an Associate Professor of Medicine and Pediatrics and the Director of the Adult Neurofibromatosis Clinical Program in the Division of Oncology, Sarcoma Section at Washington University School of Medicine. Dr. Hirbe has been awarded a grant for her study titled "Define the Global Dysregulation That Occurs in Cells with Chr8Q Gain (MPNST)." 

About Her Study: 

Malignant peripheral nerve sheath tumors (MPNST) occur in 8 to 13% of individuals with neurofibromatosis type 1 (NF1) and often develop from plexiform neurofibromas. Approximately 50% of MPNST cases may recur, and the only curative treatment option requires surgery, which is not always feasible. Thus, there is a desperate need for improved therapies. While the loss of the NF1 gene is a necessary factor in MPNST formation, additional genetic changes, such as mutations in TP53, CDKN2A, and EED/SUZ12, also contribute to tumor development.  Additionally 80% of MPNST have an extra copy of chromosome 8.

Key findings from Dr. Hirbe's studies include: 

1. An extra copy of chromosome 8 is associated with poorer survival rates. 

2. There are 58 genes on chromosome 8 that impact survival of cancer cells. 

3. A laboratory system has been developed to replicate NF1-related chromosome changes. 

4. Drug responses may vary based on chromosome 8 status. These findings suggest that alterations on chromosome 8 may enhance MPNST survival and could aid in identifying effective treatments. 

The lab aims to define the global dysregulation occurring in cells with Chr8q gain and assess treatment responses to targeted therapies based on chromosome 8 status. This exciting work will inform the development of clinical trials which will be based on the genetic makeup of this aggressive cancer.

Read more about Dr. Hirbe here.

Dr. Hirbe’s research is supported by the NF Network's Project Dream grant program, launched in 2023 to fund early-stage, high-potential NF research. Our initial study has already produced exciting results, but the urgency has intensified.

With the recent reduction of essential funding for NF research, the very scientific momentum and clinical trial infrastructure that countless families rely on are at risk.

We urge you to join us in supporting Project Dream. Your generosity can bring us closer to vital breakthroughs in neurofibromatosis research. To learn more about how you can make a difference, please visit http://weblink.donorperfect.com/project-dream.