Benign peripheral nerve sheath tumor of the extremities: a retrospective analysis and review of the literature

Year 2023, Volume: 40 Issue: 4, 717 - 720, 03.01.2024

Tolgahan Cengiz , Şafak Aydın Şimşek , Hüseyin Sina Coşkun , Nevzat Dabak

Abstract

Peripheral nerve sheath tumors (PNSTs) are a group of primary neurogenic tumors. Most of these tumors are benign. The malignant transformation could occur, especially in large tumors and those associated with neurofibromatosis type 1 (NF 1). These tumors can cause pain, nerve damage and loss of function in the affected extremities. Treatment usually includes surgery to remove the tumor or observation. Benign peripheral nerve sheath tumors (BPNST) include schwannoma, neurofibroma, and perineurium. This study aims to evaluate the demographic data, admission complaints, diagnosis, and surgical treatment steps of the patients who were operated on due to peripheral nerve sheath tumors and to compare the data with the literature. In this study, a retrospective examination was made of patients who presented with complaints of pain and swelling and were treated surgically in the Orthopedics and Traumatology Clinic of the 3rd Step University Hospital and diagnosed with BPNST between January 2010 and April 2022. The study included 107 patients diagnosed with benign peripheral nerve sheath tumors. 56 of 107 patients were excluded because the tumor was around the spine, head and neck. A total of 51 patients, 31 schwannomas, 12 neurofibroma, three traumatic neuromas, three plexiform schwannoma and two plexiform neurofibroma, were evaluated, and the data were analyzed. The most common subtype of BPNST was determined to be schwannoma and followed by neurofibroma. Benign nerve tumors originate from the nerve sheath. The most common are schwannoma, followed by neurofibromas. Typically surgical excision is recommended for BPNTS causing pain, swelling or other symptoms. Following surgical treatment, temporary postoperative symptoms could occur, like pain, paraesthesia, and sensory or motor function of the affected nerve. A biopsy should be planned when malignancy is suspected, and an experienced team should diagnose and treat these patients.

Keywords

Schwannoma, Neurofibroma, Peripheral nerve sheath tumor, Benign

References

• 1. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, et al. The 2007 WHO classification of tumours of the central nervous system. Acta neuropathologica. 2007;114(2):97-109.
• 2. Kilitci A, Asan Z. Periferik Sinir Kılıfı Tümörleri: 76 Olgunun Klinikopatolojik Değerlendirmesi. Sakarya Tıp Dergisi.9(2):337-41.
• 3. Slomiany MG, Dai L, Bomar PA, Knackstedt TJ, Kranc DA, Tolliver L, et al. Abrogating drug resistance in malignant peripheral nerve sheath tumors by disrupting hyaluronan-CD44 interactions with small hyaluronan oligosaccharides. Cancer research. 2009;69(12):4992-8.
• 4. Desai KI. The surgical management of symptomatic benign peripheral nerve sheath tumors of the neck and extremities: an experience of 442 cases. Neurosurgery. 2017;81(4):568-80.
• 5. Gosk J, Gutkowska O, Mazurek P, Koszewicz M, Ziółkowski P. Peripheral nerve tumours: 30-year experience in the surgical treatment. Neurosurgical review. 2015;38(3):511-21.
• 6. Montano N, D'Alessandris QG, D'Ercole M, Lauretti L, Pallini R, Di Bonaventura R, et al. Tumors of the peripheral nervous system: analysis of prognostic factors in a series with long-term follow-up and review of the literature. Journal of neurosurgery. 2016;125(2):363-71.
• 7. Adani R, Baccarani A, Guidi E, Tarallo L. Schwannomas of the upper extremity: diagnosis and treatment. La Chirurgia degli organi di movimento. 2008;92(2):85-8.
• 8. Donner TR, Voorhies RM, Kline DG. Neural sheath tumors of major nerves. Journal of neurosurgery. 1994;81(3):362-73.
• 9. Salla JT, Johann ACBR, Garcia BG, Aguiar MCF, Mesquita RA. Retrospective analysis of oral peripheral nerve sheath tumors in Brazilians. Brazilian oral research. 2009;23:43-8.
• 10. Val‐Bernal JF, Figols J, Vázquez‐Barquero A. Cutaneous plexiform schwannoma associated with neurofibromatosis type 2. Cancer. 1995;76(7):1181-6.
• 11. Murphey MD, Rhee JH, Lewis RB, Fanburg-Smith JC, Flemming DJ, Walker EA. Pigmented villonodular synovitis: radiologic-pathologic correlation. Radiographics. 2008;28(5):1493-518.
• 12. Lin J, Martel W. Cross-Sectional Imaging of Peripheral Nerve Sheath.
• 13. Artico M, Cervoni L, Wierzbicki V, D'andrea V, Nucci F. Benign neural sheath tumours of major nerves: characteristics in 119 surgical cases. Acta neurochirurgica. 1997;139(12):1108-16.
• 14. Wasa J, Nishida Y, Tsukushi S, Shido Y, Sugiura H, Nakashima H, et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. American Journal of Roentgenology. 2010;194(6):1568-74.
• 15. Guha D, Davidson B, Nadi M, Alotaibi NM, Fehlings MG, Gentili F, et al. Management of peripheral nerve sheath tumors: 17 years of experience at Toronto Western Hospital. Journal of neurosurgery. 2018;128(4):1226-34.
• 16. Andrea A, Roberto B, Carlo B, Maria CC, Mauro G, Pietro R. Schwannoma of the foot: report of four cases and literature review. Acta Bio Medica: Atenei Parmensis. 2019;90(Suppl 1):214.