XL Zhu, HT Wong, WS Poon, KW Chik, KW Chiu

Abstract

Medulloblastoma is one of the most frequent intracranial tumours of children. Advances in the surgical and adjuvant management have significantly improved the outcome of children with medulloblastoma. The 5-year survival rate of 30% in 1960s has risen to 70% now. Modern management of medulloblastoma emphasises: 1) aggressive surgery for tumour resection and release of hydrocephalus; 2) postoperative intensive adjuvant therapy. Chemotherapy is the choice for young children while radiotherapy is delayed until they reach three years of age or disease progress. Chemotherapy is used in conjunction with standard-dose radiotherapy for high-risk children, and with reduced-dose for average-risk children. The treatment of patients with recurrent disease remains a major challenge. Studies are still ongoing for further improving the survival rate while minimising treatment-induced morbidity.

成神經管細胞瘤是兒童最常見的顱內腫瘤之一。外科手術及輔助治療的進步顯著地改善了患成神經管細胞瘤兒童的預後身體狀況。5 年生存率從 1960 年的 30% 上升到現在的 70%。成神經管細胞瘤的新式治療強調：（1）積極的外科手術將腫瘤全部或次全切除以及腦積水的解除。（2）手術後的悉心輔助治療。患病幼兒選擇化療，而患病兒童達到 3 歲或疾病呈進行性進展時放療亦被選用。對高危兒化療結合標準劑量的放療，而標危兒則化療結合減少劑量的放療。再發患者的治療是一個主要挑戰。進一步改善存活率及減少因治療誘導的發病率是未來正在研究的課題。