高度原发性小脑肿瘤的治疗。

IF 0.8 4区 医学 Q3 MEDICINE, GENERAL & INTERNAL Journal of the Pakistan Medical Association Pub Date : 2024-11-01 DOI:10.47391/JPMA.24-92
Zanib Javed, Raheema Sadiq, Muhammad Shahzad Shamim
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引用次数: 0

摘要

小脑高级别胶质瘤(cHGG)在成人中并不常见,只占所有高级别胶质瘤的1%左右。这些肿瘤在流行病学、分子特征和患者年龄方面与幕上高级别胶质瘤(sHGG)不同。小脑高级别胶质瘤患者通常较年轻,神经纤维瘤病1(NF1)突变、非典型RAS突变和H3K27M突变的发生率较高。标准治疗包括手术切除,然后进行化疗和放疗。最近的研究强调了小脑肿瘤和幕上肿瘤之间的遗传差异,新的治疗方法针对特定的分子异常。由于cHGG独特的肿瘤环境,免疫疗法的疗效有限,因此需要进一步研究以改进这些罕见肿瘤的治疗策略。
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Management of high grade primary cerebellar tumours.

Cerebellar high-grade gliomas (cHGG) are uncommon in adults, making up only about 1% of all high-grade gliomas. These tumours differ from supratentorial high-grade gliomas (sHGG) in terms of epidemiology, molecular traits, and the age of the patients. cHGG patients are typically younger and show a higher frequency of neurofibromatosis 1 (NF1) mutations, atypical RAS mutations, and H3K27M mutations. Standard treatment includes surgical resection followed by chemotherapy and radiation. Recent studies emphasize the genetic differences between cerebellar and supratentorial tumours, with new treatments targetting specific molecular abnormalities. Immunotherapy has shown limited effectiveness due to the unique tumour environment in cHGG, and further research is required to improve treatment strategies for these rare tumours.

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来源期刊
CiteScore
1.10
自引率
11.10%
发文量
983
审稿时长
3-6 weeks
期刊介绍: Primarily being a medical journal, JPMA publishes scholarly research focusing on the various fields in the areas of health and medical education. It publishes original research describing recent advances in health particularly clinical studies, clinical trials, assessments of pathogens of diagnostic importance, medical genetics and epidemiological studies. Review articles highlighting importance of various issues in the domain of public health, drug research and medical education are also accepted. As a leading journal of South Asia, JPMA remains cognizant of the recent advances in the rapidly growing fields of biomedical sciences, it invites and encourages scholars to write short reviews and invited editorials on the emerging issues. We particularly aim to promote health standards of developing countries by encouraging manuscript submissions on issues affecting the public health and health delivery services.
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