VIVA-Asia Rare Tumor Board – 16 May 2024

Case Presentation by Dr Mo, Zhu
A male child, aged 10 years and 8 months now, began experiencing high fever and loss of consciousness at 1 year and 8 months old. Local hospital examination revealed a pineocytoma (WHO GRADE I), which was treated with surgery and chemotherapy, resulting in remission. During a follow-up at 3 years and 4 months, another CNS tumor was discovered in the fourth ventricle. After surgical removal, the child was referred to University of Hong Kong – Shenzhen Hospital, where the pathological diagnosis was Atypical Teratoid/Rhabdoid Tumor (ATRT, WHO GRADE IV). Following the surgery, the child underwent chemotherapy and radiotherapy according to the ATRT04-DDCMV protocol. Up to now, the child has experienced multiple recurrences, including 5 surgeries and 6 radiotherapy sessions. He is currently undergoing maintenance chemotherapy with the MEMMAT protocol.

Novel Therapy for Atypical Teratoid/Rhabdoid Tumor (ATRT) by Dr Ku, Dennis
ATRT exhibits a less favorable prognosis in comparison to other CNS embryonal tumors, even with the application of intensive treatment. However, with recent advancements in the molecular subgrouping of ATRT and enhanced understanding at the molecular level, novel treatment approaches and clinical trials are rapidly emerging. This presentation aims to provide a concise overview of the innovative therapies being developed for ATRT.