Hannah Flower1, Pasquele Gallo1,2. 1. Medical School, University of Edinburgh, Edinburgh, UK. 2. Western General Hospital and Royal Hospital Sick Children, Edinburgh, UK.
Abstract
Introduction: Neurofibromatosis type 1 is an autosomal dominant tumour syndrome with an increased risk of developing central nervous system neoplasms, mostly benign low-grade gliomas involving the optic pathway and the brainstem. High-grade astrocytomas or glioblastoma multiforme (GBM) are rare. Cerebellar GBMs are rarer still, only seven cases NF1 patients have been reported Case description: We report a case of a cerebellar GBM in a 25-year-old male with NF1 who survived 18 months from the initial diagnosis without surgical debulking but only chemo and radiotherapy. Conclusion: A literature review found that the best outcomes were achieved in NF1 patients who didn't undergo gross surgical resection. Possible reasons and future directions are discussed.
Introduction: Neurofibromatosis type 1 is an autosomal dominant tumour syndrome with an increased risk of developing central nervous system neoplasms, mostly benign low-grade gliomas involving the optic pathway and the brainstem. High-grade astrocytomas or glioblastoma multiforme (GBM) are rare. Cerebellar GBMs are rarer still, only seven cases NF1patients have been reported Case description: We report a case of a cerebellar GBM in a 25-year-old male with NF1 who survived 18 months from the initial diagnosis without surgical debulking but only chemo and radiotherapy. Conclusion: A literature review found that the best outcomes were achieved in NF1patients who didn't undergo gross surgical resection. Possible reasons and future directions are discussed.
Authors: Luc Bauchet; Jacques Guyotat; Thiébaud Picart; David Meyronet; Johan Pallud; Chloé Dumot; Philippe Metellus; Sonia Zouaoui; Moncef Berhouma; François Ducray Journal: J Cancer Res Clin Oncol Date: 2021-01-05 Impact factor: 4.553