Abstract: Neurofibromatosis type I (NF1) is an autosomal dominant inherited disorder caused by the NF1 gene mutation leading to abnormal regulation of the RAS pathway. Its most representative feature, neurofibroma, may present as an infiltrating plexiform neurofibroma (pNF) that invades the orbital and periorbital tissues. Scientists have therefore proposed a new term called orbital-periorbital plexiform neurofibromas (OPPN) for this type of pNF. The complexity of OPPN poses significant challenges to medical professionals. Existing treatment options include surgical treatment and targeted drug therapy. Surgical intervention can alleviate the patient's condition to a certain extent, but the overall effect remains unsatisfactory. Targeted drug therapy has developed rapidly in recent years, and some drug clinical trials have also shown promising results. In this paper, the mechanism, clinical features and treatment progress of OPPN were reviewed.

Key words: Neurofibromatosis type 1, 　Plexiform neurofibroma, 　Orbital tumor, 　MEK1/2 inhibitor