Abstract Details

Title Prolonged Survival in Adult Patients with Neurofibromatosis Type I and Recurrent High Grade Gliomas Treated with Bevacizumab

Topic Neuro-oncology

Presentation(s) P01 - (-)

Poster/Presentation
Number 098

Objective

Background NF1 mutations are present in a subset of sporadic glioblastomas with mesenchymal gene expression profiles. To our knowledge, treatment outcomes of adult NF1 patients with recurrent HGGs have not been described previously.

Design/Methods This is a series of 5 consecutive, adult NF1 patients with recurrent HGGs. Available clinical and pathologic information are reported.

Results 4 patients met clinical criteria for NF1, 1 patient had suspected segmental NF1. Median age at HGG diagnosis was 32 years (range 27-59). 3 patients had glioblastomas, 1 gliosarcoma, and 1 infiltrating hypothalamic tumor (no biopsy). 2 tumors had mutational profiling: 1 BRAF V600E mutation and 1 PIK3CA mutation were found. The anatomic locations were as follows: 2 brainstem, 1 cerebellar, 1 frontal lobe, and 1 hypothalamic. 2 patients had received prior CNS radiotherapy (1 pilocytic astrocyoma, 1 leukemia). 4 patients received concurrent chemoradiotherapy with temozolomide. 1 patient received 6 cycles of carboplatin and etoposide upfront and proton radiotherapy at first recurrence. All patients received bevacizumab at standard dosing and schedule at tumor recurrence. The median number of 4-week cycles of bevacizumab was 15 (range 13-39). The median overall survival from initial diagnosis is 27.3 months (range 15-93). The median overall survival from initiation of bevacizumab at recurrence is 20.3 months (range 10-71). 1 patient stopped bevacizumab after 39 cycles due to cardiac ischemia. 3 patients are alive and progression free. 2 patients died: 1 from pneumonia and 1 due to tumor progression.

Conclusions In this case series, adult patients with recurrent HGGs had prolonged overall and post-recurrence survival. These results suggest bevacizumab may be an effective therapy in NF1 patients with recurrent HGGs. Based on these observations, evaluation of bevacizumab responses in sporadic glioblastomas with NF1 mutations is warranted.

Authors/Disclosures
Brett J. Theeler, MD, FAAN (Uniformed Services University of the Health Sciences) PRESENTER	Dr. Theeler has nothing to disclose.
Benjamin Ellezam, MD (CHU Sainte-Justine - Pathologie)	No disclosure on file
	No disclosure on file
Monica E. Loghin, MD (UT MD ANderson Cancer Center)	Dr. Loghin has nothing to disclose.
John F. De Groot, MD (UC San Francisco Medical Center)	An immediate family member of Dr. De Groot has received personal compensation for serving as an employee of Ziopharm Oncology. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Celldex. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Deciphera Pharmaceuticals. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for AbbVie. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for FivePrime Therapeutics, Inc.. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for GW Pharma. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Carthera. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Eli Lilly. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Kadmon. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Boston Biomedical Inc.. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Taiho Pharmaceuticals. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Kairos Venture Investments. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Syneos Health. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Monteris. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Agios. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Mundipharma Research. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for GenomiCare. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Blue Earth Diagnostics. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Del Mar Pharmaceuticals. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Insightec. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Voyager Therapeutics, Inc.. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Merck & Co.. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Tocagen. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bioasis Technologies, Inc.. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for ResTORbio, Inc.. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Roche. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Celldex. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Foundation Medicine, Inc.. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novogen. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Deciphera. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Astrazeneca. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Insys Therapeutics. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck & Co.. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Eli Lily. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novella Clinical. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Karyopharm Therapeutics. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Blue Earth Diagnostics. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kiyatec. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Vanquish Oncology. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Orsenix, Insightec. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Prelude Therapeutics. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Debiopharm Therapeutics, Inc.. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janssen Global Services, LLC. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for VBL Therapeutics. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novella. Dr. De Groot has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for VBI Vaccines, Inc.. An immediate family member of Dr. De Groot has received stock or an ownership interest from Ziopharm Oncology. An immediate family member of Dr. De Groot has received stock or an ownership interest from Gilead. The institution of Dr. De Groot has received research support from Carthera. The institution of Dr. De Groot has received research support from HaiHe Pharma. The institution of Dr. De Groot has received research support from Taiho Pharma.
Alasdair Coles, MD, PhD (University of Cambridge)	Dr. Coles has nothing to disclose.

��ɫ��

��ɫ��