医学
神经纤维瘤病
横纹肌肉瘤
神经纤维蛋白1
神经母细胞瘤
发病机制
癌症研究
肉瘤
周围神经鞘恶性肿瘤
癌症
病理
激酶
内科学
生物
细胞生物学
遗传学
细胞培养
作者
Hilde Brems,Eline Beert,Thomy de Ravel,Eric Legius
标识
DOI:10.1016/s1470-2045(09)70033-6
摘要
Neurofibromatosis type 1 (NF1) is a familial tumour syndrome. Malignant tumours can arise in the nervous and non-nervous system in either childhood or adulthood, with malignant peripheral nerve sheath tumours being most common. Rhabdomyosarcoma and neuroblastoma are paediatric neoplasms that are more common in children with NF1 than in those without the syndrome. Gastrointestinal stromal tumours, somatostatinomas, breast cancer, and phaeochromocytomas are seen in adults with NF1. Several pathways are thought to be involved in the development of tumours associated with NF1: rat sarcoma viral oncogene homologue (RAS)–mitogen activated protein kinase (MAPK), mammalian target of rapamycin (mTOR), and P21 protein (Cdc42/Rac)–activated kinase 1 (PAK1). New insights into the pathogenesis of these tumours will lead to a better understanding of tumour origin and development and will hopefully allow the discovery of new and specific treatments.
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