SURGICAL TACTICS AND RESULTS OF TREATMENT OF HEAD NEUROFIBROMAS: A CLINICAL EXPERIENCE
DOI:
https://doi.org/10.11603/1811-2471.2025.v.i3.15555Keywords:
type 1 neurofibromatosis, plexiform tumor, microsurgery, mitogen-activated protein kinase inhibitors, 3D planning, reconstructive procedures, intraoperative monitoring, quality of lifeAbstract
SUMMARY. The aim – to improve the effectiveness of surgical treatment of patients with head neurofibromas by systematizing clinical experience in treating such patients in the context of type 1 neurofibromatosis (NF1), with an emphasis on the choice of surgical tactics, reconstruction methods, and the use of innovative technologies, especially regarding the combination of surgical and medical approaches to improve functional and aesthetic results.
Material and Methods. Current achievements and a clinical case of a patient with a head neurofibroma who underwent treatment at the Valikhnovski Surgery Institute were shown. Clinical examination, imaging methods (magnetic resonance imaging, Dopplerography), morphological confirmation, intraoperative neurophysiological monitoring, surgical methods (resection, microsurgery, laser ablation), reconstructive techniques (tissue expansion, free flaps), quality of life assessment, and statistical analysis of results at a p<0.05 level were presented.
Results. Surgical intervention led to sustained improvement. The main complications included neurological deficits, recurrences, and reconstruction complications (expander extrusion, flap necrosis). The integration of 3D visualization, mitogen-activated protein kinase inhibitors (selumetinib), and personalized planning significantly improves treatment effectiveness. The presented clinical case demonstrates the successful application of a comprehensive approach with radical tumor removal, reconstruction of the defect with local tissues, and long-term remission without recurrence.
Conclusions. Surgical treatment of head neurofibromas in patients with type 1 neurofibromatosis is an effective method that requires an individualized approach, taking into account the anatomical location and morphology of the tumor. Reconstructive interventions using tissue expansion and free flaps allow achieving high functional and aesthetic results even with large defects. The integration of intraoperative neuromonitoring, 3D visualization, and targeted therapy increases the safety of surgical interventions and expands the indications for the treatment of complex forms of neurofibromas. Prospects for further research include conducting multicenter prospective studies to standardize surgical tactics and reconstructive algorithms in patients with craniofacial neurofibromas, as well as studying the long-term effectiveness of combined treatment.
References
Wang J, Fu J, Zhou Y, Gao D, Qing J, Yang G. Global research trends in cutaneous neurofibromas: A bibliometric analysis from 2003 to 2022. Skin Res Technol. 2024 Jan 26;30(2):e13595. DOI: 10.1111/srt.13595. DOI: https://doi.org/10.1111/srt.13595
Adil A, Koritala T, Munakomi S, Singh AK. Neurofibromatosis Type 1. In: StatPearls. StatPearls Publishing; 2023 [cited 2025 Apr 28]. Available from: https://www.ncbi.nlm.nih.gov/sites/books/NBK459358/
Poplausky D, Young JN, Tai H, Rivera-Oyola R, Gulati N, Brown RM. Dermatologic Manifestations of Neurofibromatosis Type 1 and Emerging Treatments. Cancers. 2023 Jan;15(10):2770. DOI: 10.3390/cancers15102770. DOI: https://doi.org/10.3390/cancers15102770
Latham K, Buchanan EP, Suver D, Gruss JS. Neurofibromatosis of the head and neck: classification and surgical management. Plast Reconstr Surg. 2015 Mar;135(3):845–55. DOI: 10.1097/PRS.0000000000000960. DOI: https://doi.org/10.1097/PRS.0000000000000960
Hsu C-K, Denadai R, Chang C-S, Yao C-F, Chen Y-A, Chou P-Y, et al. The Number of Surgical Interventions and Specialists Involved in the Management of Patients with Neurofibromatosis Type I: A 25-Year Analysis. J Pers Med. 2022 Apr 1;12(4):558. DOI: 10.3390/jpm12040558. DOI: https://doi.org/10.3390/jpm12040558
Cacchione A, Carboni A, Lodi M, Vito RD, Carai A, Marrazzo A, et al. Congenital Craniofacial Plexiform Neurofibroma in Neurofibromatosis Type 1. Diagnostics. 2021 Feb;11(2):218. DOI: 10.3390/diagnostics11020218. DOI: https://doi.org/10.3390/diagnostics11020218
Ikuta K, Nishida Y, Sakai T, Koike H, Ito K, Urakawa H, et al. Surgical Treatment and Complications of Deep-Seated Nodular Plexiform Neurofibromas Associated with Neurofibromatosis Type 1. J Clin Med. 2022 Sep 26;11(19):5695. DOI: 10.3390/jcm11195695. DOI: https://doi.org/10.3390/jcm11195695
Ota M, Nobeyama Y, Asahina A. Real-world Settings for the Surgical Treatment of Neurofibroma in Patients with Neurofibromatosis Type 1. JMA J. 2024 Apr 15;7(2):205–12. DOI: 10.31662/jmaj.2023-0161. DOI: https://doi.org/10.31662/jmaj.2023-0161
Chung J-H, Kim HJ, Kang H-C, Kim IH, Lee JH. Long-term outcomes of radiotherapy for inoperable benign soft tissue tumors in the skull base or head. Radiat Oncol J. 2025 Mar;43(1):49–54. DOI: 10.3857/roj.2024.00493. DOI: https://doi.org/10.3857/roj.2024.00493
Längle G, Gohritz A, Gstöttner C, Harnoncourt L, Platzgummer H, Azizi AA, et al. [Plastic surgical treatment of neurofibromatosis type 1]. Chirurgie (Heidelb). 2025 May;96(5):394–404. DOI: 10.1007/s00104-024-02232-5. DOI: https://doi.org/10.1007/s00104-024-02232-5
Pal A, Bhola N, Agarwal A, Ghavat C, Jannawar G. Neurofibroma of Face: A Challenge in Aesthetic Reconstruction. International Journal of Anatomy Radiology and Surgery. 2021;10(3):SC01–3. DOI: 10.7860/JCDR/2021/ 47955/2662. DOI: https://doi.org/10.7860/IJARS/2021/47955.2662
Hivelin M, Wolkenstein P, Lepage C, Valeyrie-Allanore L. Facial Aesthetic Unit Remodeling Procedure for Neurofibromatosis Type 1 Hemifacial Hypertrophy: Report on 33 Consecutive Adult Patients. Plastic & Reconstructive Surgery. 2010;125(4):1197–207. DOI: 10.1097/ PRS.0b013e3181d180e9. DOI: https://doi.org/10.1097/PRS.0b013e3181d180e9
Bin Abdul Halim WMA, Bin Mat Hassan S, Bt Awang M, Abdullah MAH. Malignant Transformation of Plexiform Neurofibroma Due to Neglected Giant Soft Tissue Swelling of the Back: A Case Report. Cureus. 2024 Jul 4;16(7):e63807. DOI: 10.7759/cureus.63807. DOI: https://doi.org/10.7759/cureus.63807
Nehete LS, Sharma R, Singh PR, Gupta S. Rapidly growing diffuse neurofibroma of the scalp with calvarial defect in a young woman: A rare entity. Surgical Neurology International. 2020 Oct 2;11:313. DOI: 10.25259/SNI_213_2020. DOI: https://doi.org/10.25259/SNI_213_2020
Kaam DN, Nepomuceno AC, Da-Silva LC, Aquino JLBD, Staut JG, Morano FG, et al. Scalp reconstruction with expanded flap. Revista Brasileira de Cirurgia Plástica (RBCP) – Brazilian Journal of Plastic Sugery. 2021;36(1):96–9. DOI: 10.5935/2177-1235.2021RBCP0018. DOI: https://doi.org/10.5935/2177-1235.2021RBCP0018
Uygur F, Chang DW, Crosby MA, Skoracki RJ, Robb GL. Free flap reconstruction of extensive defects following resection of large neurofibromatosis. Ann Plast Surg. 2011 Oct;67(4):376–81. DOI: 10.1097/SAP. 0b013e3182085b13. DOI: https://doi.org/10.1097/SAP.0b013e3182085b13
Martinez DC, Badhey A, Cervenka B, Zender C, Tang A, Patil Y. Surgical Techniques for Head and Neck Reconstruction in the Vessel-Depleted Neck. Facial Plast Surg. 2020 Dec;36(6):746–52. DOI: 10.1055/s-0040-1721108. DOI: https://doi.org/10.1055/s-0040-1721108
Anastasova V, Krasteva E, Kiskinov P, Ivanova K, Georgiev A, Dachev D, et al. Our experience using tissue expansion in reconstructive surgery. Folia Medica. 2025 Mar 21;67(2):e137928. DOI: 10.3897/folmed.67.e137928. DOI: https://doi.org/10.3897/folmed.67.e137928
Wu D, Yan C, Gao H, Liu Z, Sun Y, Xu L, et al. Tissue expansion techniques in reconstructive surgery: a 10-year bibliometric analysis. Ann Transl Med. 2023 Mar 15;11(5):204. DOI: 10.21037/atm-22-3643. DOI: https://doi.org/10.21037/atm-22-3643
Strode MA, Lohman RF, Salerno KE, Kane JM. Tissue Expander Use for Reconstruction of Scalp Soft Tissue Sarcoma Wide Resection Defects. Plastic Surgery Case Studies. 2020 Jan 1;6:2513826X20903503. DOI: 10.1177/2513826X20903503. DOI: https://doi.org/10.1177/2513826X20903503
Singhal D, Chen Y-C, Seselgyte R, Chen PK-T, Chen Y-R. Craniofacial neurofibromatosis and tissue expansion: long-term results. J Plast Reconstr Aesthet Surg. 2012 Jul;65(7):956–9. DOI: 10.1016/j.bjps.2011.11.034. DOI: https://doi.org/10.1016/j.bjps.2011.11.034
Raposio G, Raposio E. Scalp reconstruction by tissue expansion: tips and tricks. Acta Biomed. 2022;93(5):e2022313. DOI: 10.23750/abm.v93i5.13393.
Gahankari D, McCombe A, Meares T. Early rapid tissue expansion of a free microvascular flap in head-neck reconstruction as salvage option: a case report. Australas J Plast Surg. 2024 Apr 9;7(1):1–4. DOI: 10.34239/ajops.88851. DOI: https://doi.org/10.34239/ajops.88851
Azzi JL, Thabet C, Azzi AJ, Gilardino MS. Complications of tissue expansion in the head and neck. Head Neck. 2020 Apr;42(4):747–62. DOI: 10.1002/hed.26017. DOI: https://doi.org/10.1002/hed.26017
Langdell HC, Taskindoust M, Levites HA, Mateas C, Sergesketter AR, Kaplan SJ, et al. Systematic Review of Tissue Expansion: Utilization in Non-breast Applications. Plast Reconstr Surg Glob Open. 2021 Jan;9(1):e3378. DOI: 10.1097/GOX.0000000000003378. DOI: https://doi.org/10.1097/GOX.0000000000003378
Jiang M, Liu X, Li D, Baigong F, Shen C. Risk factors for complications of tissue expansion: An updated systematic review and meta-analysis. Surgery. 2025 Mar 13;181:109282. DOI: 10.1016/j.surg.2025.109282. DOI: https://doi.org/10.1016/j.surg.2025.109282
Cîrstea A-I, Berteșteanu Șerban VG, Vrînceanu D, Dumitru M, Bejenaru PL, Simion-Antonie CB, et al. Perspectives in Using Multiple Flaps Reconstructions for Advanced Head and Neck Tumors (Scoping Review). Medicina. 2024 Aug;60(8):1340. DOI: 10.3390/medicina60081340. DOI: https://doi.org/10.3390/medicina60081340
Mohanty AJ, DeVahl J, Kutz WJ, Rozen SM. Neurofibromatosis Type II and Facial Paralysis: Clinical Evaluation and Management. Plast Reconstr Surg. 2024 Feb 1;153(2):415e–23e. DOI: 10.1097/PRS.0000000000010561. DOI: https://doi.org/10.1097/PRS.0000000000010561
Yoshinaga A, Tsuge I, Saito S, Morimoto N. Orbital/Periorbital Plexiform Neurofibromas: Classification and Surgical Strategies for a Better Outcome. Plastic and Reconstructive Surgery - Global Open. 2023 Jul;11(7):e5108. DOI: 10.1097/GOX.0000000000005108. DOI: https://doi.org/10.1097/GOX.0000000000005108
As’adi K, Ahmadi S, Mirsalehi M. Surgical treatment of craniofacial cleft and orbital hypertelorism: a single-center cohort study. Neurosurg Focus. 2025 Jan 1;58(1):E4. DOI: 10.3171/2024.10.FOCUS24573. DOI: https://doi.org/10.3171/2024.10.FOCUS24573
Ching RC, Wall SA, Johnson D. Neoforehead Remodelling Techniques for Fronto-Orbital Advancement and Remodelling in the Oxford Craniofacial Unit: Lateral Remodelling and a Novel Central Self-Stabilizing S-Osteotomy Technique. J Craniofac Surg. 2023 Feb 1;34(1):20–8. DOI: 10.1097/SCS.0000000000008859. DOI: https://doi.org/10.1097/SCS.0000000000008859
Fan X-Q, Lin M, Li J, Zhou H-F, Fu Y. [Removal and plastic reconstructive surgery of orbital neurofibroma]. Zhonghua Yi Xue Za Zhi. 2007 Dec 11;87(46):3264–7.
Nawres BM, Murtadha MBE, Maytham YA-T. New methods for craniofacial bone reconstruction: review. JPTCP. 2023 Jan 1;30(4):241–8. DOI: 10.47750/jptcp.2023.30.04.023. DOI: https://doi.org/10.47750/jptcp.2023.30.04.023