Head and Neck Plexiform Neurofibroma

• Involvement of Cranial Nerve Ganglion as an Independent Risk Factor for Malignant Transformation of Head and Neck Plexiform Neurofibromas in Neurofibromatosis Type 1
• Plexiform Schwannoma of the Foot: A Case Report
• Plexiform Neurofibroma Versus Parotid Tumour - A Challenge in Management
• Pediatric Spindle Cell Tumor in Head and Neck Region: A Case Series
• Facial Diffuse Plexiform Neurofibroma-associated Mandibular Deformities: Surgical Interventions and Monitoring of Treatment Results in a Patient for Over 40 Years
• Selumetinib for symptomatic, inoperable plexiform neurofibromas in pediatric patients with neurofibromatosis type 1: the first single-center real-world case series in Japan
• A single-center case study series assessing the effect of selumetinib use in patients with neurofibromatosis-related plexiform neurofibromas
• Clinical experiences in precision treatment of giant plexiform neurofibromas of head, face, and neck

Plexiform neurofibromas, in the head and neck region, pose unique clinical and surgical challenges due to their proximity to vital neurovascular structures and potential for disfigurement and functional impairment.

• Occurs in ~30–50% of patients with NF1.
• Typically appears in childhood or adolescence.
• Head and neck PNFs represent a high-risk anatomical subtype due to morbidity and potential for malignant transformation.

• Visible mass with possible facial asymmetry or disfigurement.
• Pain, paresthesias, or motor deficits depending on nerve involvement.
• Airway obstruction, dysphagia, or dysphonia if deep cervical or cranial nerves are involved.
• Often infiltrative, non-encapsulated, and progressive.
• May extend along cranial nerves, brachial plexus, or into the skull base.

• Risk of malignant peripheral nerve sheath tumor (MPNST) is estimated at 8–13% in NF1 patients with PNF.
• Recent evidence suggests cranial nerve ganglion involvement is an independent risk factor for transformation.

→ See: Gu Y, et al. *Plast Reconstr Surg*. 2025 Jul 8. DOI: 10.1097/PRS.0000000000012302.

• Surgical resection remains the mainstay but is often incomplete due to infiltration and risk of neurovascular injury.
• Debulking for functional or cosmetic purposes.
• Emerging therapies:
  • Selumetinib (MEK inhibitor) approved for symptomatic, inoperable PNFs in children with NF1.
• Close monitoring with serial MRI and neurological evaluation.

Head and Neck Plexiform Neurofibroma Prognosis