Faden Daniel L, Asthana Saurabh, Tihan Tarik, DeRisi Joseph, Kliot Michel
Department of Otolaryngology-Head and Neck Surgery, University of California San Francisco, San Francisco, California, United States of America.
Helen Diller Comprehensive Cancer Center, University of California San Francisco, San Francisco, California, United States of America.
PLoS One. 2017 Jan 18;12(1):e0170348. doi: 10.1371/journal.pone.0170348. eCollection 2017.
The growth behaviors of cutaneous neurofibromas in patients with Neurofibromatosis type 1 are highly variable. The role of the germline NF1 mutation, somatic NF1 mutation and mutations at modifying loci, are poorly understood. We performed whole exome sequencing of three growing and three non-growing neurofibromas from a single individual to assess the role of acquired somatic mutations in neurofibroma growth behavior. 1-11 mutations were identified in each sample, including two deleterious NF1 mutations. No trends were present between the types of somatic mutations identified and growth behavior. Mutations in the HIPPO signaling pathway appeared to be overrepresented.
1型神经纤维瘤病患者皮肤神经纤维瘤的生长行为高度可变。种系NF1突变、体细胞NF1突变以及修饰位点突变的作用尚不清楚。我们对来自同一个体的三个生长中的和三个未生长的神经纤维瘤进行了全外显子组测序,以评估获得性体细胞突变在神经纤维瘤生长行为中的作用。每个样本中鉴定出1-11个突变,包括两个有害的NF1突变。所鉴定的体细胞突变类型与生长行为之间没有趋势性关联。HIPPO信号通路中的突变似乎过度存在。