Surgical Innovations in Neurofibromatosis Tumors: Nerve Preservation Strategies, Functional Mapping, and Emerging Technologies

Background: Neurofibromatosis (NF) Type 1 and Type 2 are autosomal dominant tumor predisposition syndromes characterized by the development of nerve sheath tumors intimately associated with critical neural structures. Surgical management has historically been constrained by the high risk of neurological morbidity, particularly facial nerve paralysis, hearing loss, sensory deficits, and motor deficits. However, over the past decade, the operative paradigm has shifted from radical resection toward function-preserving strategies supported by technological innovation. Objective: To synthesize contemporary surgical innovations that enable maximal safe resection of neurofibromatosis-associated tumors while prioritizing nerve preservation and long-term functional outcomes. Methods: This narrative review integrates current evidence on intraoperative neurophysiological monitoring, predictive analytics, minimally invasive and robotic-assisted techniques, adjunct molecular therapies, and advanced digital surgical planning technologies, including three-dimensional modeling, virtual reality simulation, and augmented reality navigation. Results: Multimodal intraoperative neurophysiological monitoring, including electromyography, motor evoked potentials, somatosensory evoked potentials, and brainstem auditory evoked potentials, has significantly improved the detection and prevention of neural injury during NF tumor resection. Predictive models and machine learning algorithms now allow preoperative and intraoperative risk stratification for facial nerve outcomes in vestibular schwannoma surgery with high diagnostic accuracy. Minimally invasive and robotic-assisted approaches have reduced surgical morbidity in complex plexiform neurofibromas by enhancing visualization and facilitating fascicle-sparing dissection. The integration of MEK inhibitors, particularly selumetinib, introduces a multimodal framework in which medical debulking complements precision surgery. Advanced planning technologies, including patient-specific three-dimensional printing, virtual rehearsal platforms, and augmented reality navigation, have further enhanced anatomical understanding and intraoperative accuracy. Conclusions: The management of neurofibromatosis-associated tumors has evolved into a technologically integrated, multimodal discipline centered on functional preservation. The convergence of real-time neurophysiological monitoring, artificial intelligence-driven risk stratification, minimally invasive robotics, multimodal advanced imaging, and digital surgical planning represents a paradigm shift toward precision neurosurgery. Future progress will depend on homogenizing surgical innovation with molecular therapies to optimize long-term neurological function and quality of life in this complex patient population.

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Journal
Medical Sciences
Published
2026-10-27
DOI
https://doi.org/10.3390/medsci14070716
Primary Topic
Neurofibromatosis and Schwannoma Cases
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article

Surgical Innovations in Neurofibromatosis Tumors: Nerve Preservation Strategies, Functional Mapping, and Emerging Technologies

José E. León-Rojas, Joaquin Fiallo Arroyo
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Medical Sciences
Neurofibromatosis and Schwannoma Cases
article

Surgical Innovations in Neurofibromatosis Tumors: Nerve Preservation Strategies, Functional Mapping, and Emerging Technologies

José E. León-Rojas, Joaquin Fiallo Arroyo
article en

Abstract

Background: Neurofibromatosis (NF) Type 1 and Type 2 are autosomal dominant tumor predisposition syndromes characterized by the development of nerve sheath tumors intimately associated with critical neural structures. Surgical management has historically been constrained by the high risk of neurological morbidity, particularly facial nerve paralysis, hearing loss, sensory deficits, and motor deficits. However, over the past decade, the operative paradigm has shifted from radical resection toward function-preserving strategies supported by technological innovation. Objective: To synthesize contemporary surgical innovations that enable maximal safe resection of neurofibromatosis-associated tumors while prioritizing nerve preservation and long-term functional outcomes. Methods: This narrative review integrates current evidence on intraoperative neurophysiological monitoring, predictive analytics, minimally invasive and robotic-assisted techniques, adjunct molecular therapies, and advanced digital surgical planning technologies, including three-dimensional modeling, virtual reality simulation, and augmented reality navigation. Results: Multimodal intraoperative neurophysiological monitoring, including electromyography, motor evoked potentials, somatosensory evoked potentials, and brainstem auditory evoked potentials, has significantly improved the detection and prevention of neural injury during NF tumor resection. Predictive models and machine learning algorithms now allow preoperative and intraoperative risk stratification for facial nerve outcomes in vestibular schwannoma surgery with high diagnostic accuracy. Minimally invasive and robotic-assisted approaches have reduced surgical morbidity in complex plexiform neurofibromas by enhancing visualization and facilitating fascicle-sparing dissection. The integration of MEK inhibitors, particularly selumetinib, introduces a multimodal framework in which medical debulking complements precision surgery. Advanced planning technologies, including patient-specific three-dimensional printing, virtual rehearsal platforms, and augmented reality navigation, have further enhanced anatomical understanding and intraoperative accuracy. Conclusions: The management of neurofibromatosis-associated tumors has evolved into a technologically integrated, multimodal discipline centered on functional preservation. The convergence of real-time neurophysiological monitoring, artificial intelligence-driven risk stratification, minimally invasive robotics, multimodal advanced imaging, and digital surgical planning represents a paradigm shift toward precision neurosurgery. Future progress will depend on homogenizing surgical innovation with molecular therapies to optimize long-term neurological function and quality of life in this complex patient population.

Medical SciencesVol. 14(7)
Universidad de Las Américas (EC)
Good health and well-being
Openalex Percentile: Top 14%
Neurofibromatosis and Schwannoma Cases
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