Head and Neck Plexiform Neurofibroma [Neurosurgery Wiki]

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====== Head and Neck Plexiform Neurofibroma ======

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[[Plexiform neurofibroma]]s, 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.


===== Epidemiology =====
  * 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.

===== Clinical Presentation =====
  * **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.



===== Malignant Potential =====
  * 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.

===== Treatment =====
  * **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.

===== Prognosis =====

[[Head and Neck Plexiform Neurofibroma Prognosis]]