Abstract
Abstract INTRODUCTION Neurofibromatosis type 1 (NF1) is a rare tumor suppressor syndrome that predisposes to nervous system tumors. The optimal treatment of gliomas in adults with NF1 is uncertain. Specifically, little is known about the effectiveness or toxicity of radiation therapy (RT) in this population although it is standard of care for progressive low grade glioma (LGG) or high grade glioma (HGG) in general. We evaluated the tolerability of RT and time to progression (TTP) after completion of RT in people with NF1 diagnosed with glioma in adulthood. METHODS Charts were reviewed for patients with a confirmed NF1 syndrome, LGG or HGG, > 18 years old, receiving RT and followed at Johns Hopkins University between 2009–2019. Secondary outcomes were overall survival (OS) from diagnosis and RT associated toxicity. Progression was defined radiographically; toxicity was graded by CTCAE. RESULTS Eight people (6 males) with median age of 36.5 years (range, 20–63) received RT at median of 56.7Gy either at the time of diagnosis (N=4) or at recurrence (N=4). Three patients (37.5%) had LGG; five (62.5%) had HGG. Tumor locations were brainstem (6) and temporal lobe (2). All patients received concurrent chemotherapy with RT; temozolomide (7) or bevacizumab (1). At median follow-up of 28.3 months (0.9–90.2) from RT completion, 5/8 patients had progression. Of those who progressed, median TTP was 12.7 months (2.5–26.6). The longest period of disease control was 28.9 months. One and two-year OS rates were 87% and 32%, respectively. RT associated toxicities were within the expected range. CONCLUSIONS Overall disease control after RT was variable, with some examples of extraordinary duration of disease control, and RT toxicities were within expected range. Collaborative efforts to examine a larger cohort of adults with NF1 associated gliomas are needed to define optimal care, but these observations suggest RT is a feasible modality.
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