Application of multimodal intraoperative neurophysiological monitoring technology in neurofibromatosis type 1 related peripheral nerve tumor surgery_Chinese Journal of Reparative and Reconstructive Surgery

Authors：

ZHOU Weiyun ¹ , ZENG Yu ¹ , OUYANG Huoniu ¹ , ZHANG Wenchuan ¹ , WANG Zhichao ² ,  WU Yiwei ¹

1. Department of Neurosurgery, Shanghai Ninth People’s Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, 200011, P. R. China;
2. Department of Plastic and Reconstructive Surgery, Shanghai Ninth People’s Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, 200011, P. R. China;

Corresponding author：

WU Yiwei, Email: ywwu115098@163.com

Keywords：

Neurofibromatosis type 1; multimodal intraoperative neurophysiological monitoring; peripheral nerve tumor

DOI：

10.7507/1002-1892.202406065

Video：

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Objective To summarize application effect and clinical experience of multimodal intraoperative neurophysiological monitoring (IONM) technology in the surgery of neurofibromatosis type 1 (NF1) related peripheral nerve tumors. Methods A retrospective study was conducted on NF1 patients, who admitted between January 2019 and December 2023 and treated with peripheral nerve tumor resection surgery assisted by multimodal IONM technology. There were 49 males and 45 females. The age ranged from 5 to 78 years, with an average of 33.7 years. Tumor morphological classification included 71 cases of nodular type, 13 cases of diffuse type, and 10 cases of mixed type. Target tumors were distributed in craniofacial region (47 cases), neck (11 cases), trunk (12 cases), and limbs (24 cases). Preoperatively, 44 cases had no obvious neurological symptoms, while the remaining patients had neurological symptoms, including 15 cases of visual impairment, 5 cases of hearing impairment, 16 cases of somatic movement disorders, and 31 cases of somatic sensory disorders, of which 7 cases had more than one symptom. IONM plans were selected based on the relevant nerves and adjacent important structures of the target tumor, including visual evoked potential (17 cases), somatosensory evoked potential (44 cases), motor evoked potential (88 cases), and electromyogram (94 cases).Results All surgeries were successfully completed. Ninety-three patients underwent total/near total resection and 1 patient underwent palliative resection. Pathological examination showed 80 cases of neurofibroma and 14 cases of malignant peripheral nerve sheath tumors. Complications included 2 cases of hematoma and 3 cases of incision infection. All patients were followed up 3-61 months (median, 15 months). During follow-up, no significant changes in neurological symptoms or tumor recurrence were found. Among the patients with preoperative visual impairment, there were 14 cases with no improvement in symptoms and 1 with improvement after surgery. Among the patients with somatic movement disorders, there were 11 cases with no improvement in symptoms, 3 cases with improvement, 2 cases with aggravation, 4 newly onset cases, and 1 case with significant impact on daily life after surgery. Among the patients with somatic sensory disorders, there were 17 cases with no improvement in symptoms, 14 cases with improvement, and 13 newly onset cases. The patients with hearing impairment showed improvement after surgery. Conclusion The clinical manifestations of NF1 related peripheral nerve tumors are complex. Multimodal IONM technology can provide real-time detection of nerve provocation and damage. Surgical treatment with multimodal IONM technology is safe and can reduce complications.

Citation： ZHOU Weiyun, ZENG Yu, OUYANG Huoniu, ZHANG Wenchuan, WANG Zhichao, WU Yiwei. Application of multimodal intraoperative neurophysiological monitoring technology in neurofibromatosis type 1 related peripheral nerve tumor surgery. Chinese Journal of Reparative and Reconstructive Surgery, 2024, 38(10): 1202-1207. doi: 10.7507/1002-1892.202406065 Copy

1.	Mautner VF, Asuagbor FA, Dombi E, et al. Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1. Neuro Oncology, 2008, 10(4): 593-598.
2.	Waggoner DJ, Towbin J, Gottesman G, et al. Clinic-based study of plexiform neurofibromas in neurofibromatosis 1. Am J Med Genet, 2000, 92(2): 132-135.
3.	Gross AM, Wolters PL, Dombi E, et al. Selumetinib in children with inoperable plexiform neurofibromas. N Engl J Med, 2020, 382(15): 1430-1442.
4.	Voelker R. A new treatment for children with neurofibromatosis type 1. JAMA. 2020, 323(19): 1887. doi: 10.1001/jama.2020.7157.
5.	Fisher JM, Blakeley JO, Weiss BD, et al. Management of neurofibromatosis type 1-associated plexiform neurofibromas. Neuro Oncology, 2022, 24(11): 1827-1844.
6.	王智超, 李青峰. Ⅰ型神经纤维瘤病临床诊疗专家共识 (2021 版). 中国修复重建外科杂志, 2021, 35(11): 1384-1395.
7.	Bashiri FA, Hundallah K, Abukhaled M, et al. Diagnosis and management of neurofibromatosis type 1 in Arabian Gulf Cooperation Council Region: challenges and recommendations. Front Oncol, 2024, 14: 1323176. doi: 10.3389/fonc.2024.1323176.
8.	Armstrong AE, Belzberg AJ, Crawford JR, et al. Treatment decisions and the use of MEK inhibitors for children with neurofibromatosis type 1-related plexiform neurofibromas. BMC Cancer, 2023, 23(1): 553. doi: 10.1186/s12885-023-10996-y.
9.	梁汉生, 冯艺. 术中神经电生理监测的现状及展望. 中华医学杂志, 2015, 95(21): 1646-1647.
10.	Garozzo D. Peripheral nerve tumors in neurofibromatosis 1: An overview on management and indications for surgical treatment in our experience. Neurol India, 2019, 67(Supplement): S38-S44.
11.	Zelenski NA, Oishi T, Shin AY. Intraoperative neuromonitoring for peripheral nerve surgery. J Hand Surg (Am), 2023, 48(4): 396-401.
12.	Rich BS, Brown EG, Rothstein DH, et al. The utility of intraoperative neuromonitoring in pediatric surgical oncology. J Pediatr Surg, 2023, 58(9): 1708-1714.
13.	Nelson CN, Dombi E, Rosenblum JS, et al. Safe marginal resection of atypical neurofibromas in neurofibromatosis type 1. J Neurosurg, 2019, 133(5): 1516-1526.
14.	Rijken BF, Veelen-Vincent ML, Mathijssen IM. Sphenoid dysplasia in patients with neurofibromatosis type 1: Clinical features and imaging findings including cerebrospinal fluid alterations. Eur J Paediatr Neurol, 2023, 42: 28-33.
15.	Anetakis KM, Dolia JN, Desai SM, et al. Last electrically well: intraoperative neurophysiological monitoring for identification and triage of large vessel occlusions. J Stroke Cerebrovasc Dis, 2020, 29(10): 105158. doi: 10.1016/j.jstrokecerebrovasdis.2020.105158.
16.	Matsuo R, Kamouchi M, Inoue T, et al. Cerebral infarction due to carotid occlusion caused by cervical vagal neurilemmoma: report of a case. Stroke, 2002, 33(5): 1428-1431.

1. Mautner VF, Asuagbor FA, Dombi E, et al. Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1. Neuro Oncology, 2008, 10(4): 593-598.
2. Waggoner DJ, Towbin J, Gottesman G, et al. Clinic-based study of plexiform neurofibromas in neurofibromatosis 1. Am J Med Genet, 2000, 92(2): 132-135.
3. Gross AM, Wolters PL, Dombi E, et al. Selumetinib in children with inoperable plexiform neurofibromas. N Engl J Med, 2020, 382(15): 1430-1442.
4. Voelker R. A new treatment for children with neurofibromatosis type 1. JAMA. 2020, 323(19): 1887. doi: 10.1001/jama.2020.7157.
5. Fisher JM, Blakeley JO, Weiss BD, et al. Management of neurofibromatosis type 1-associated plexiform neurofibromas. Neuro Oncology, 2022, 24(11): 1827-1844.
6. 王智超, 李青峰. Ⅰ型神经纤维瘤病临床诊疗专家共识 (2021 版). 中国修复重建外科杂志, 2021, 35(11): 1384-1395.
7. Bashiri FA, Hundallah K, Abukhaled M, et al. Diagnosis and management of neurofibromatosis type 1 in Arabian Gulf Cooperation Council Region: challenges and recommendations. Front Oncol, 2024, 14: 1323176. doi: 10.3389/fonc.2024.1323176.
8. Armstrong AE, Belzberg AJ, Crawford JR, et al. Treatment decisions and the use of MEK inhibitors for children with neurofibromatosis type 1-related plexiform neurofibromas. BMC Cancer, 2023, 23(1): 553. doi: 10.1186/s12885-023-10996-y.
9. 梁汉生, 冯艺. 术中神经电生理监测的现状及展望. 中华医学杂志, 2015, 95(21): 1646-1647.
10. Garozzo D. Peripheral nerve tumors in neurofibromatosis 1: An overview on management and indications for surgical treatment in our experience. Neurol India, 2019, 67(Supplement): S38-S44.
11. Zelenski NA, Oishi T, Shin AY. Intraoperative neuromonitoring for peripheral nerve surgery. J Hand Surg (Am), 2023, 48(4): 396-401.
12. Rich BS, Brown EG, Rothstein DH, et al. The utility of intraoperative neuromonitoring in pediatric surgical oncology. J Pediatr Surg, 2023, 58(9): 1708-1714.
13. Nelson CN, Dombi E, Rosenblum JS, et al. Safe marginal resection of atypical neurofibromas in neurofibromatosis type 1. J Neurosurg, 2019, 133(5): 1516-1526.
14. Rijken BF, Veelen-Vincent ML, Mathijssen IM. Sphenoid dysplasia in patients with neurofibromatosis type 1: Clinical features and imaging findings including cerebrospinal fluid alterations. Eur J Paediatr Neurol, 2023, 42: 28-33.
15. Anetakis KM, Dolia JN, Desai SM, et al. Last electrically well: intraoperative neurophysiological monitoring for identification and triage of large vessel occlusions. J Stroke Cerebrovasc Dis, 2020, 29(10): 105158. doi: 10.1016/j.jstrokecerebrovasdis.2020.105158.
16. Matsuo R, Kamouchi M, Inoue T, et al. Cerebral infarction due to carotid occlusion caused by cervical vagal neurilemmoma: report of a case. Stroke, 2002, 33(5): 1428-1431.

Chinese Journal of Reparative and Reconstructive Surgery

Application of multimodal intraoperative neurophysiological monitoring technology in neurofibromatosis type 1 related peripheral nerve tumor surgery

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