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Volumetric measurement techniques for assessment of cutaneous neurofibromas:A review

Chinese Journal of Plastic and Reconstructive Surgery 2024年第2期6卷 83-86页

作者： Xinyu Wang Jiayi Xu Yun Zhu Chengjiang Wei Zhichao Wang School of Medicine Shanghai Jiao Tong University Department of Plastic and Reconstructive Surgery Shanghai Ninth People's Hospital Shanghai Jiao Tong University School of Medicine

cutaneous neurofibroma(cNF) is a prevalent clinical manifestation of neurofibromatosis type 1, significantly affecting the well-being and quality of life of the affected individuals. The adoption of reliable and reproducible volumetric measurement techniques is essential for precisely evaluating tumor burden and plays a critical role in the development of effective treatments for cNF. This study focuses on widely used volumetric measurement techniques, including vernier calipers, ultrasound, computed tomography, magnetic resonance imaging, and three-dimensional scanning imaging. It outlines the merits and drawbacks of each technique in assessing the cNF load, providing an overview of their current applications and ongoing research advancements in this domain.

关键词： cutaneous neurofibroma Tumor volume measurement Vernier caliper Ultrasound Computed tomography Magnetic resonance imaging Three-dimensional scanning imaging

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Targeting the extracellular matrix for NF1-associated neurofibroma treatment

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Chinese Journal of Plastic and Reconstructive Surgery 2024年第2期6卷 87-93页

作者： Chunhui Jiang NHC Key Laboratory of Cell Transplantation First Affiliated Hospital of Harbin Medical University

neurofibromatosis type 1(NF1) is one of the most common genetic disorders that predisposes patients to benign and malignant tumors of the peripheral nervous system. Plexiform and cutaneous neurofibromas are NF1-associated benign tumors. Despite their benign nature, they can cause tremendous morbidity in patients with NF1. Therapeutic drug options are limited to the MEK inhibitor, selumetinib, which is the only approved drug for pediatric patients with plexiform neurofibromas. Antifibrotic strategies have substantial therapeutic potential for NF1-associated neurofibromas. This review discusses the fibrotic features of plexiform and cutaneous neurofibromas focusing on the pathological composition of the extracellular matrix. It also highlights the core pathways implicated in the biochemical and biophysical regulation of the extracellular matrix remodeling in tumor imitation and progression. Finally, this review provides a brief outlook on how exploring novel vulnerabilities residing in the aberrant extracellular matrix and their underlying pathways can benefit the treatment of NF1-associated neurofibromas.

关键词： neurofibromatosis type 1 cutaneous neurofibroma Plexiform neurofibroma Fibrosis Extracellular matrix Basement membrane

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