Neurofibromatosis Type 1 (NF1) Related Tumors (2nd Edition)
A special issue of Cancers (ISSN 2072-6694). This special issue belongs to the section "Tumor Microenvironment".
Deadline for manuscript submissions: 30 June 2026 | Viewed by 323
Special Issue Editors
Interests: NF1-associated tumors; plexiform neurofibromas; glioblastoma multiforme
Special Issues, Collections and Topics in MDPI journals
Interests: NF1 tumor; pediatric brain tumors; spinal cord tumors
Special Issues, Collections and Topics in MDPI journals
Special Issue Information
Dear Colleagues,
This Special Issue is a continuation of our previous Special Issue, “Neurofibromatosis Type 1 (NF1) Related Tumors” (https://www.mdpi.com/journal/cancers/special_issues/MKJGT4K1HG).
Neurofibromatosis Type 1 (NF1) is a cancer predisposition syndrome, with afflicted patients developing benign and/or malignant tumors at distinct developmental stages. The pathognomonic feature is the development of nerve sheath tumors, cutaneous neurofibromas, and plexiform neurofibromas, with the latter capable of transformation into malignant peripheral nerve sheath tumor (MPNST), an incurable sarcoma with metastatic potential. Central nervous system (CNS) tumors are also frequently encountered in children, including optic pathway gliomas (OPGs) and brainstem low-grade gliomas (LGGs), while an increased incidence of high-grade gliomas (HGGs) in adolescents and young adults compared to the general population has also been reported. With the identification of NF1 and co-operating driver alterations through collaborative multi-omics studies and the development of genetically engineered mouse models that accurately recapitulate these tumor phenotypes, therapies targeting the underlying molecular pathophysiology (i.e., RAS signaling and downstream effectors) for neurofibromatosis type 1 are becoming available and have demonstrated success in benign plexiform neurofibromas and optic pathway gliomas. In addition, patients with NF1 show increased risk for leukemia, sarcomas, breast cancer, pheochromocytoma and gastrointestinal stromal tumors, among others. NF1-associated tumors demonstrate significant tumor heterogeneity, an enriched tumor microenvironment, and versatile epigenetic regulations that create significant challenges in the development of new drugs and treatment strategies, often contributing to worse outcomes relative to sporadic NF1 wild-type tumors. Recent advances in the NF1 field demonstrate stem-like tumor populations driving heterogeneity, characterize the immune microenvironment and inflammatory signatures, and elucidate the crystal structure of neurofibromin. These new angles may further characterize NF1-associated tumors and inspire new treatment perspectives. Clinical trials evaluating targeted therapy (i.e., MEK inhibitors) versus standard approaches are ongoing and may potentially result in a paradigm shift in the treatment of benign NF1 tumors, while combinatorial approaches are likely to be required for the management of high-grade tumors.
In this Special Issue, we aim to summarize, explore, and characterize the features of NF1-related tumors, covering both basic and (pre)clinical aspects to advance our understanding of targeting these complex diseases.
We look forward to receiving your contributions.
Dr. Daochun Sun
Dr. Sameer Farouk Sait
Guest Editors
Manuscript Submission Information
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Keywords
- NF1
- tumorigenesis
- cancer stem cells
- targeted therapy
- tumor microenvironment
- epigenetics
- case report
- metastasis
- clinical trials
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