[Treatment and progress of cutaneous neurofibroma]

Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2022 Sep 15;36(9):1064-1071. doi: 10.7507/1002-1892.202205072.
[Article in Chinese]

Abstract

Objective: To summarize current widely-used therapies for cutaneous neurofibroma (cNF) and related research progress.

Methods: Based on extensive investigation of domestic and foreign research, the existing treatment of cNF, including the indications, effectiveness and trials of targeted drugs were reviewed.

Results: cNF is a hallmark feature of neurofibromatosis type 1 and has a dramatic negative impact on patient appearance and quality of life. At present, there is no standard management of cNF. Invasive treatment is a commonly-used treatment. Surgical removal gives excellent cosmetic results, but it is difficult for multiple tumors; CO2 laser ablation, laser photocoagulation, electro-drying, and radiofrequency ablation are effective in treating lots of cNF at one time. Although fast and effective, these therapies can lead to depigmentation, hyperpigmentation, or extensive scarring. There is no targeted drug approval for cNF, and a series of studies have been carried out on the Ras-MEK pathway, Ras-mTOR pathway, receptor tyrosine kinase, et al.

Conclusion: The treatment of cNF has developed rapidly in recent years and has broad prospects, but the individualization and precision of the treatment still needs further clinical research.

目的: 总结目前临床常用的皮肤型神经纤维瘤(cutaneous neurofibroma,cNF)治疗方案以及相关研究进展。.

方法: 广泛查阅cNF治疗相关文献,对现有治疗方案的适应证及其疗效,以及药物临床研究进展进行总结分析。.

结果: cNF是1型神经纤维瘤病的一种标志性特征,对患者外观和生活质量产生极大负面影响。目前该病治疗缺乏统一标准。侵入性治疗是临床常用治疗方案,其中手术治疗疗效确切,但不适用于大量瘤体患者;CO2激光消融、激光光凝、电干燥、射频消融等方法能一次性治疗大量瘤体,兼具处理速度快和治疗效果好的特点,但会导致色素减退、色素沉着或大量瘢痕形成。靶向药物展现出了广阔应用前景,针对Ras-MEK通路、Ras-mTOR通路、受体酪氨酸激酶等方面的药物临床研究正在开展,尚未批准上市。.

结论: 近年来,cNF的治疗发展迅速,具有广阔前景,但各治疗方案适应证有待进一步临床研究明确。.

Keywords: Neurofibromatosis type 1; cutaneous neurofibroma; research progress; treatment.

Publication types

  • Review

MeSH terms

  • Carbon Dioxide
  • Humans
  • Mitogen-Activated Protein Kinase Kinases
  • Neurofibroma* / metabolism
  • Neurofibroma* / pathology
  • Neurofibroma* / therapy
  • Protein-Tyrosine Kinases
  • Quality of Life
  • Skin Neoplasms* / pathology
  • Skin Neoplasms* / therapy
  • TOR Serine-Threonine Kinases

Substances

  • Carbon Dioxide
  • Protein-Tyrosine Kinases
  • TOR Serine-Threonine Kinases
  • Mitogen-Activated Protein Kinase Kinases

Grants and funding

国家自然科学基金资助项目(82102344、82172228)