👤 Vanessa Angelica Suntoro

Dysembryoplastic neuroepithelial tumours (DNETs) are rare with only a few hundred cases reported in literature. The long-term natural history of paediatric DNETs is still poorly understood. We present a rare case of DNET with recurrent tumour bleeds over 26 years of follow-up and provide a brief literature review of similar events. The patient is a 30-year-old Chinese male who presented with right-sided seizures since 3 years old. Initial neuroimaging was suspicious of left fronto-parietal glioma and was conservatively managed due to its location near the motor strip and concerns of potential surgical complications. Over the follow-up period, the patient suffered three bleeds. Following the third bleed, tumour resection was performed under intraoperative motor mapping, with near total resection. Intraoperatively, the tumour involved the post-central gyrus, with histological findings suggestive of low-grade glioma with FGFR1 alteration, in keeping with DNET. The latest neuroimaging showed no new haemorrhages or infarcts. Features of small residual tumour around the tumour cavity were noted. The patient is currently well with marked reduction of seizure episodes. Our report provides new insight into the long-term natural history of DNET and adds value to limited existing literature of similar cases. Show less