Mushtaq Naureen, Resham Shahzadi, Shamim Shahzad, Qureshi Bilal Mazhar, Riaz Quratulain, Bouffet Eric
Department of Oncology, Aga Khan University Hospital, Karachi, Pakistan.
Department of Paediatrics and Child Health, Aga Khan University Hospital, Karachi, Pakistan.
J Pak Med Assoc. 2020 Nov;70(11):2007-2016. doi: 10.5455/JPMA.293142.
Medulloblastoma is the most common malignant brain tumour in children and is a major cause of mortality and morbidity, particularly in low- and middle-income countries. It has been risk-stratified on the basis of clinical (age, metastasis and extent of resection) and histological subtypes (classic, desmoplastic and anaplastic). However, recently medulloblastoma has been sub-grouped by using a variety of different genomic approaches, such as gene expression profiling, micro-ribonucleic acid profiling and methylation array into 4 groups, namely Wingless, Sonic hedgehog, Group 3 and Group 4. This new sub-grouping has important therapeutic and prognostic implications. After acute leukaemia, brain tumour is the second most common malignancy in the paediatric age group. The improvement in outcome of acute lymphoblastic leukaemia in low- and middle-income countries reflects the relative simplicity of diagnostic procedures and management. Unlike leukaemia, the management of brain tumours requires a complex multidisciplinary approach, including neuro-radiologists, neurosurgeons with a paediatric expertise, neuropathologists, radiation oncologists and neuro-oncologists. In addition, the equipment required for the diagnosis (magnetic resonance imaging scan, histological, molecular and genetic techniques) and the management (operating room, radiation facilities) is a limiting factor in countries with limited resources. In Pakistan, there are very few centres able to treat children with brain tumours. The current literature review was planned to provide an update on the management of this tumour.
髓母细胞瘤是儿童最常见的恶性脑肿瘤，是导致死亡和发病的主要原因，在低收入和中等收入国家尤其如此。它已根据临床因素（年龄、转移和切除范围）和组织学亚型（经典型、促纤维增生型和间变型）进行了风险分层。然而，最近髓母细胞瘤通过使用多种不同的基因组方法，如基因表达谱分析、微小核糖核酸谱分析和甲基化阵列，被分为4组，即Wingless组、Sonic hedgehog组、3组和4组。这种新的分组具有重要的治疗和预后意义。在急性白血病之后，脑肿瘤是儿童年龄组中第二常见的恶性肿瘤。低收入和中等收入国家急性淋巴细胞白血病治疗结果的改善反映了诊断程序和管理相对简单。与白血病不同，脑肿瘤的治疗需要复杂的多学科方法，包括神经放射科医生、具有儿科专业知识的神经外科医生、神经病理学家、放射肿瘤学家和神经肿瘤学家。此外，诊断所需的设备（磁共振成像扫描、组织学、分子和基因技术）以及治疗所需的设备（手术室、放疗设施）在资源有限的国家是一个限制因素。在巴基斯坦，能够治疗儿童脑肿瘤的中心非常少。本次文献综述旨在提供关于这种肿瘤治疗的最新情况。
