Sectoral schwannomatosis of the sciatic nerve: Clinical and Surgical Aspects
https://doi.org/10.18705/2311-4495-2025-12-6-576-583
Abstract
Schwannomas are the most common benign tumors of peripheral nerves and spinal nerve roots. Sectoral schwannomatosis, in turn, is a rare condition (0.58 cases per 1 million people) characterized by multiple tumors affecting a single nerve trunk, with no pathological lesions in other anatomical areas. Unlike systemic forms of neurofibromatosis, sectoral schwannomatosis typically lacks cutaneous manifestations and bilateral vestibular nerve involvement, making diagnosis particularly challenging. Over the past decade, only a few cases of sectoral schwannomatosis involving the sciatic nerve have been reported in the literature. Herein, we present a rare clinical case of a female patient with sectoral schwannomatosis of the sciatic nerve and its branches requiring surgical intervention. Neuroimaging (MRI and ultrasound) revealed multiple mass lesions extending along the sciatic nerve in the distal third of the thigh, as well as along the tibial nerve in the proximal third of the lower leg and the popliteal region. The total length of the involved nerve segment reached up to 20 cm, with tumors exhibiting heterogeneous structure and pronounced vascularization on Doppler imaging. The clinical presentation featured intractable neuropathic pain. No tumors were found in other nerves. The patient underwent surgical removal of five tumor nodules using direct nerve stimulation (intraoperative neuromonitoring), which allowed for clear differentiation between functionally significant nerve fascicles and tumor tissue, thereby preventing their damage. Postoperatively, nerve function was fully preserved, and pain regressed to 1 point on the visual analogue scale within the first five days. A review of the relevant literature is also provided. Given the rarity of this pathology, accumulation of clinical experience is essential; therefore, each identified case of sectoral schwannomatosis warrants a detailed description.
About the Authors
A. V. GorodninaRussian Federation
Angelina V. Gorodnina, MD, PhD, Neurosurgeon
191014; 12 Mayakovskaya str.; St. Petersburg
Competing Interests:
The authors declare no conflict of interest
D. A. Sitovskaya
Russian Federation
Darya A. Sitovskaya, MD, PhD, Pathologist
St. Petersburg
Competing Interests:
The authors declare no conflict of interest
A. Yu. Orlov
Russian Federation
Andrey Yu. Orlov, MD, PhD, DSc, Neurosurgeon, Head of the Laboratory
Research Laboratory of Neurosurgery of the Spine and Peripheral Nervous System
St. Petersburg
Competing Interests:
The authors declare no conflict of interest
References
1. Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. The lancet neurology. 2007;6(4):340–351. doi: 10.1016/S1474-4422(07)70075-3
2. Warade A, Roy R, Pattankar S, et al. Segmental schwan-nomatosis of the lower extremity — a case series. Neurology India. 2022;70(5):2132–2136. doi: 10.4103/0028-3886.359176
3. WHO Classification of Tumours Editorial Board. Central nervous system tumours. Lyon: International Agency for Research on Cancer. WHO Classification of Tumours Series. 5<sup>th</sup> ed., vol. 6. 2021.
4. Andreani L, Ipponi E, Ruinato AD, et al. Peripheral schwannomas of the tibial nerve: surgical results in a case series. Acta Biomedica. 2022;14;93(1):e2022085. doi: 10.23750/abm.v93i1.11782
5. Erdoğan F, Say F, Barış YS. Schwannomatosis of the sciatic nerve: a case report. British Journal of Neurosurgery. 2023;37(1):100–103. doi: 10.1080/02688697.2021.1950628
6. Shishiba T, Niimura M, Ohtsuka F, Tsuru N. Multiple cutaneous neurilemmomas as a skin manifestation of neurilemmomatosis. Journal of the American Academy Dermatology. 1984;10:744–754. doi: 10.1016/s0190-9622(84)70089-2
7. Schraepen C, Donkersloot P, Duyvendak W, et al. What to know about schwannomatosis : a literature review. British Journal of Neurosurgery. 2022;36(2):171–174. doi: 10.1080/02688697.2020.1836323
8. Kwon NY, Oh HM, Ko YJ. Multiple lower extremity mononeuropathies by segmental schwannomatosis: a case report. Annals of rehabilitation medicine. 2015;26;39(5):833–837. doi: 10.5535/arm.2015.39.5.83
9. Plotkin SR, Wick A. Neurofibromatosis and schwannomatosis. Seminars in neurology. 2018;38 (1):73–85. doi: 10.1055/s-0038-1627471
10. MacCollin M, Chiocca EA, Evans DG, et al Diagnostic criteria for schwannomatosis. Neurology. 2005;64:1838–1845. doi: 10.1212/01.WNL.0000163982.78900.AD
11. Plotkin SR, Blakeley JO, Evans DG, et al. Update from the 2011 international schwannomatosis workshop: from genetics to diagnostic criteria. American Journal of Medical Genetics. 2013;161:405–416. doi: 10.1002/ajmg.a.35760
12. Alaidarous A, Parfait B, Ferkal S, et al. Segmental schwannomatosis: characteristics in 12 patients. Orphanet journal of rare diseases. 2019;22(14):207. doi: 10.1186/s13023-019-1176-4
13. Pilavaki M, Chourmouzi D, Kiziridou A, et al. Imaging of peripheral nerve sheath tumors with pathologic correlation : pictorial review. European Journal of Radiology. 2004;52:229–239. doi: 10.1016/j.ejrad.2003.12.001
14. Guzzi G, Ricciuti RA, Torre AD, et al. Intraoperative neurophysiological monitoring in neurosurgery. Journal of clinical medicine. 2024;17;13(10):2966. doi: 10.3390/jcm13102966
15. Huang J, Mobbs R, Teo C. Multiple schwannomas of the sciatic nerve. Journal of Clinical Neuroscience. 2003;10:391–393. doi: 10.1016/s0967-5868(03)00021-3
16. Ogose A, Hotta T, Morita T, et al. Multiple schwannomas in the peripheral nerves. The Journal of Bone and Joint Surgery. British volume. 1998;80:657–661. doi: 10.1302/0301-620x.80b4.8532
17. Yamamoto T, Maruyama S, Mizuno K. Schwannomatosis of the sciatic nerve. Skeletal Radiology. 2001;30:109–113. doi: 10.1007/s002560000310
18. Gonzalvo A, Fowler A, Cook RJ, et al. Schwannomatosis, sporadic schwannomatosis, and familial schwannomatosis: a surgical series with long-term follow-up. Journal of Neurosurgery. 2011;114:756–762. doi: 10.3171/2010.8.JNS091900
Review
For citations:
Gorodnina A.V., Sitovskaya D.A., Orlov A.Yu. Sectoral schwannomatosis of the sciatic nerve: Clinical and Surgical Aspects. Translational Medicine. 2025;12(6):576-583. (In Russ.) https://doi.org/10.18705/2311-4495-2025-12-6-576-583
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