Surgical management of plexiform neurofibromas: An imaging-based perioperative framework

Plexiform neurofibromas (PNs) are complex, infiltrative peripheral nerve sheath tumors affecting up to half of individuals with neurofibromatosis type 1 (NF1). Their diffuse, multicompartment growth, potential for disfigurement and neurologic compromise, and elevated risk of malignant peripheral nerve sheath tumor make management uniquely challenging. Optimal care hinges on accurate imaging, multidisciplinary planning, and strategic selection between complete resection and function-preserving debulking. MRI is central to perioperative assessment, with whole-body MRI supporting baseline mapping and regional MRI providing high-resolution definition for operative planning. Angiographic studies assist in identifying candidates for preoperative embolization, while FDG-PET improves detection of distinct nodular lesions and atypical neurofibromas with higher malignant potential. Preoperative embolization may reduce intraoperative hemorrhage in highly vascular PNs. Surgical indications include progressive neurologic deficits, airway or organ compromise, refractory pain, and severe disfigurement. Complete resection offers maximal oncologic control but risks permanent deficits; subtotal or debulking procedures prioritize function preservation while accepting recurrence risk. Adjuncts such as fluorescein-guided resection, intraoperative neurophysiological monitoring, and 3-dimensional planning enhance precision and safety. Reconstruction requires site-specific strategies for head and neck, truncal, and extremity lesions. Postoperatively, MRI-based surveillance is critical, particularly following subtotal resection or in high-risk anatomical locations. MEK inhibitors, including selumetinib, provide effective non-surgical therapy for progressive or unresectable PNs. Management of PNs demands individualized, multidisciplinary care. An imaging-driven approach, selective use of embolization, and incorporation of intraoperative adjuncts can optimize tumor control and functional outcomes. Prospective multicenter studies are needed to standardize imaging protocols, surgical selection criteria, and long-term outcome reporting.