Long-Term Results for a One-Stage Surgery Technique for Patients With Craniofacial Plexiform Neurofibroma

Chen, Liangbo; Guo, Chengrui; Song, Xuefei; Yan, Chenxi; Hu, Xiaojie

doi:10.1097/scs.0000000000004685

Cited by 4 publications

(2 citation statements)

References 19 publications

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“…Furthermore, PNFs may present in the form of diffuse enlargement of the affected body region (11,29,32). Current surgical experience has shown that recurrence rates for BPNSTs range from 1.3% to 54% and are higher in pediatric PNFs (29,30,32,(34)(35)(36)(37). Outcome is largely influenced by tumor histopathology, type of mutation and tumor location (34,(38)(39)(40).…”

Section: Treatment Of Pnstsmentioning

confidence: 99%

New Frontiers in Therapy of Peripheral Nerve Sheath Tumors in Patients With Neurofibromatosis Type 1: Latest Evidence and Clinical Implications

et al. 2020

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Almost all individuals with neurofibromatosis type 1 (NF1) develop peripheral nerve sheath tumors (PNSTs), mainly benign neurofibromas, however about 10% of PNSTs will undergo transformation to malignant peripheral nerve sheath tumors (MPNSTs). Surgical treatment of PNSTs has traditionally been regarded as a standard approach. The availability of new agents that target specific molecular pathways involved in the pathogenesis of PNST has led to a number of clinical trials, which resulted in increased chances for better survival and quality of life. This review presents the latest evidence and clinical implications for new therapies of PNSTs in patients with NF1 emphasizing the potential benefit from the use of Ras/MAPK pathway inhibitors, immunotherapy, chemotherapy or radiation therapy. We present evaluation of current knowledge on available treatment modalities. Neurofibromatosis type 1 (NF1) is an autosomal dominant neurocutaneous disease affecting about 1 in 3500 people worldwide (1). The hallmark clinical features of NF1 include multiple café-au-lait macules, neurofibromas, intertriginous freckling, osseous lesions, Lisch nodules and optic pathway gliomas. The disorder is typified by the presence of multisystem tumors, which carries a high risk of malignant transformation (2, 3). Nearly 100% of individuals with NF1 develop benign peripheral nerve sheath tumors (BPNSTs) and in approximately 30-50% of them atypical and plexiform neurofibromas (PNFs) are found. In about 10% of NF1 patients, neurofibromas may undergo transformation to malignant peripheral nerve sheath tumors (MPNSTs), which are highly aggressive. The differentiation between atypical neurofibroma and low grade MPNST is probably the most challenging issue in the pathology of peripheral nerve sheath tumors (PNSTs), particularly in NF1 patients. Clinically, atypical tumors often develop as extensive, slowly growing neoplasms, and pain can be a characteristic feature (4, 5). The poor response to currently available therapies underlines the need for more effective, targeted treatment methods for NF1-associated PNSTs (6, 7). In this review, we analyzed the latest evidence and clinical implications of new therapies of PNSTs in patients with NF1 emphasizing the importance of patient risk stratification to identify those who are likely to benefit from the use of Ras/MAPK pathway inhibitors, immunotherapy, chemotherapy or radiotherapy. We also analyzed the limitations of administering these therapies to all individuals with NF1-associated PNSTs together with future perspectives. Methodology PubMed searches were performed to identify potentially clinically relevant English-language studies published in the last 15 years. The searches were based on a combination of indexed terms and free text terms ("peripheral nerve sheath tumors"

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Section: Treatment Of Pnstsmentioning

confidence: 99%

New Frontiers in Therapy of Peripheral Nerve Sheath Tumors in Patients With Neurofibromatosis Type 1: Latest Evidence and Clinical Implications

et al. 2020

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“…1 They often lack function and aesthetic value, thereby negatively affecting quality of life. 2 There is no specific treatment for NF1, and clinical manifestations are treated individually. Surgery is the preferred treatment for plexiform neurofibromas, but the significant vascularization of the tumor and the consequent possibility of large intraoperative hemorrhages make the procedure difficult.…”

Section: Introductionmentioning

confidence: 99%

Surgery combined with embolization in the treatment of plexiform neurofibroma: Case report and literature review

et al. 2020

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Modified facelift in severe plexiform neurofibromatosis associated with venous vascular malformation: Case report

Mayer

Huatuco

Petersen

et al. 2023

Annales de Chirurgie Plastique Esthétique

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Long-Term Results for a One-Stage Surgery Technique for Patients With Craniofacial Plexiform Neurofibroma

Abstract: The surgical technique could achieve good surgical outcomes in both functional and cosmetic terms. Additionally, the relatively objective evaluation technique based on Mimics software could be a more convincing method for evaluating the surgical outcomes of craniofacial patients with PNF.

Cited by 4 publications

References 19 publications

New Frontiers in Therapy of Peripheral Nerve Sheath Tumors in Patients With Neurofibromatosis Type 1: Latest Evidence and Clinical Implications

New Frontiers in Therapy of Peripheral Nerve Sheath Tumors in Patients With Neurofibromatosis Type 1: Latest Evidence and Clinical Implications

Surgery combined with embolization in the treatment of plexiform neurofibroma: Case report and literature review

Modified facelift in severe plexiform neurofibromatosis associated with venous vascular malformation: Case report

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