PNR-04NOVEL MANAGEMENT OF A NEUROGLIAL HAMARTOMA/GANGLIOGLIOMA WITH CHEMOTHERAPY AND PET IMAGING: TWO CASE REPORTS

Abstract

PNR-04. NOVEL MANAGEMENT OF A NEUROGLIAL HAMARTOMA/GANGLIOGLIOMA WITH CHEMOTHERAPY AND PET IMAGING: TWO CASE REPORTS Beryl Lin1,3, Ignacio Gonzalez2, and Stacie Stapleton2,3; University of New South Wales, Sydney,NSW, Australia; All Children’s Hospital, St Petersburg, FL,USA; JohnsHopkinsUniversitySchool ofMedicine, Baltimore,MD,USA INTRODUCTION: Neuroglial hamartomas of the cerebellopontine angle (CPA) are extremely rare tumours of the CNS, with only 12 prior cases in the literature. Curative resection is not always achievable, and few studies have examined the utility of adjuvant therapies. We present two unusual cases of very symptomatic, non-excisable pontine hamartoma/gangliogliomas and a novel approach to their imaging and treatment. CASE REPORT: Patient 1 is a 9-year old boy who presented with vertigo and hearing loss. MRI revealed a 25x10mm right CPA mass. Despite aggressive subtotal resection of his hamartoma/ganglioglioma, the patient continued to develop severe headaches and tremors that significantly impacted his quality of life. Patient 2 is 9-month-old boy who presented with hemifacial spasms. EEG was normal however, MRI revealed a similar 3x3x2cm left CPA mass, proven on biopsy to be a hamartoma/ ganglioglioma. Both patients, despite stable evaluation upon MRI over several years, suffered life-impacting clinical deterioration. Notably, both lesions were persistently avid for F-18 FDG on PET. Two years after first presentation, patient 1 trialled non-standard treatment with chemotherapy. Within 6 months,hispontine lesionbecamenon-avidonPETwithcompletesymptomatic resolution. Currently, 6 months following a 2-year chemotherapy protocol, patient 1 has remained asymptomatic with excellent tumour control. Patient 2 declined chemotherapy. Despite stable evaluation on MRI, his spasms continue to worsen, now interfering with his speech and social interactions. CONCLUSION: To our knowledge, this is the first study to report successful use of PET neuroimaging and chemotherapy in neuroglial hamartomas/gangliogliomas for consideration in future management. Neuro-Oncology 18:iii7–iii15, 2016. doi:10.1093/neuonc/now067.4 #The Author(s) 2016. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.