Journal: Zhongguo xiu fu chong jian wai ke za zhi = Zhongguo xiufu chongjian waike zazhi = Chinese journal of reparative and reconstructive surgery
This article is an expert interpretation of the 2025 Chinese consensus on the full-course management of plexiform neurofibromas (PNFs) in patients with neurofibromatosis type 1 (NF1).
Key points:
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Context and rationale
NF1 is an autosomal dominant disorder caused by NF1 gene mutations, commonly complicated by PNFs, which can be disfiguring, function-threatening, or life‑threatening. The emergence of MEK inhibitors has substantially changed therapeutic options for these tumors, prompting the need for updated management guidance.
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Scope of the consensus discussed
The paper summarizes and explains recommendations from the 2025 national expert consensus issued by the Group of Neurofibromatosis of the Plastic and Reconstructive Surgery Society of the Chinese Medical Association. It focuses on:
- Diagnostic criteria and clinical evaluation of PNFs in NF1
- Screening strategies and indications for imaging
- Principles of surgical management (e.g., timing, goals, and limitations of resection)
- Role and use of systemic medical therapy, especially MEK inhibitors, in inoperable, progressive, or symptomatic PNFs
- Long-term surveillance strategies for tumor growth, complications, and malignant transformation risk
- Patient education, psychosocial support, and multidisciplinary care throughout the disease course
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Clinical relevance
The article is intended as a practical reference to help clinicians implement the consensus recommendations across the entire disease trajectory of NF1-associated PNFs, integrating surgery, targeted therapy, follow‑up, and supportive care.