Sechenov Medical Journal

Advanced search

Retrospective cohort study of morphological features of recurrent schwannomas and neurofibromas


Recurrences of benign peripheral nerves sheaths tumours (BPNST) after total resection were described in 2.6–11.0% of patients. The significance of the histological features of recurrent BPNST is still insufficiently studied.
Aim. To compare the pathomorphological features of recurrent and non-recurrent BPNST (schwannomas and neurofibromas).
Materials and methods. A retrospective assessment was made of 101 patients with BPNST with a degree of anaplasia corresponding not more than Grade I. Recurrence of BPNST developed in 13 (12.9%) cases. The study included patients with histological archive: the study group (n = 7) included patients with one or more relapses of BPNST, the control group included patients (n = 5) without relapses after surgery for 5 or more years. The main clinical characteristics were studied and histological examination was performed.
Results. There were no differences between the groups in baseline characteristics (the type of tumour (schwannoma, neurofibroma), distribution by sex, age, localization, clinical symptoms). The relapse rate among patients with neurofibromas was 8 in 3 patients vs. 6 in 5 patients with schwannomas. In all cases of recurrent schwannomas and in one of neurofibroma, the histological pattern was predominantly monophasic with rhythmic structures like Verocay bodies with underlined pattern and nuclear hyperchromasia, in contrast to the control group, represented by tumours with a mixed type of structure with uniform alternation of various histological patterns (p < 0,05). Endothelial proliferation and lymphocytic infiltration in the stroma and perivascular area were more common in the relapse group (p < 0.05). Pathomorphological signs of anaplasia: cell-nuclear polymorphism, nuclear hyperchromasia, endothelial proliferation, mitosis, as well as minor signs of anaplasia: solidization, muirization of the fascicular pattern of a tumour and apoptotic bodies were found with the same frequency in both groups. With relapse, the capsule was lost, thinned, intermittent, and sometimes invaded the surrounding tissues.
Conclusion. Tumours with the initial signs of anaplasia, such as endothelial proliferation, tendency to hypercellularity, and histological pattern with prominent Verocay bodies dominate among recurrent BPNST.

About the Authors

D. A. Murzaeva
Pavlov First Saint Petersburg State Medical University
Russian Federation


Tel.: +7 (929) 262-30-82

6–8, L’va Tolstogo str., Saint Petersburg, 197022, Russia 

Yu. M. Zabrodskaya
Polenov Neurosurgical Institute, Branch of Almazov National Medical Research Centre; Kirov Military Medical Academy
Russian Federation

 Professor, Dr. of Sci. (Medicine), Head of the Research Laboratory of Pathomorphology of the Nervous System; Senior Lecturer, Department of Pathology

12, Mayakovskogo str., Saint-Petersburg, 191014, Russia

6, Academic Lebedev str., Saint-Petersburg, 194044, Russia

A. A. Dolgushin
Polenov Neurosurgical Institute, Branch of Almazov National Medical Research Centre
Russian Federation


12, Mayakovskogo str., Saint-Petersburg, 191014, Russia

L. N. Dobrogorskaya
Polenov Neurosurgical Institute, Branch of Almazov National Medical Research Centre
Russian Federation

 laboratory assistant

12, Mayakovskogo str., Saint-Petersburg, 191014, Russia

A. Y. Orlov
Polenov Neurosurgical Institute, Branch of Almazov National Medical Research Centre
Russian Federation

Dr of Sci. (Medicine), neurosurgeon

12, Mayakovskogo str., Saint-Petersburg, 191014, Russia


1. Huang M.J., Kano H., Mousavi S.H., et al. Stereotactic radiosurgery for recurrent vestibular schwannoma after previous resection. J Neurosurg. 2017; May; 126(5): 1506–1513. Epub 2016 Jul 29. PMID: 27471891.

2. Louis D.N., Perry A., Reifenberger G., et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol. 2016 Jun; 131(6): 803–820. Epub 2016 May 9. PMID: 27157931.

3. Cheng X., Liu J., Le J., et al. Invasive intramedullary melanotic schwannoma: case report and review of the literature. Eur Spine J. 2018 Jul; 27(Suppl 3): 303–308. Epub 2017 Jul 10. PMID: 28695275.

4. Meyer A. Review and update in the diagnosis of peripheral nerve sheath tumors. Curr Opin Neurol. 2020 Oct; 33(5): 575–586. PMID: 32833749.

5. Meyer A., Billings S.D. What’s new in nerve sheath tumors. Virchows Arch. 2020 Jan; 476(1): 65–80. Epub 2019 Nov 9. PMID: 31707590.

6. Garner H.W., Wilke B.K., Fritchie K., Bestic J.M. Epithelioid schwannoma: imaging findings on radiographs, MRI, and ultrasound. Skeletal Radiol. 2019 Nov; 48(11): 1815–1820. Epub 2019 Mar 22. PMID: 30903261.

7. Ahlawat S., Blakeley J.O., Rodriguez F.J., Fayad L.M. Imaging biomarkers for malignant peripheral nerve sheath tumors in neurofibromatosis type 1. Neurology. 2019 Sep 10; 93(11): e1076–e1084. Epub 2019 Aug 8. Erratum in: Neurology. 2020 Mar 17; 94(11): 504. PMID: 31395668.

8. Perry A., Graffeo C.S., Carlstrom L.P., et al. Is there a need for a 6-month postradiosurgery magnetic resonance imaging in the treatment of vestibular schwannoma? Neurosurgery. 2020 Feb 1; 86(2): 250–256. PMID: 30980077.

9. Kalamarides M., Bernat I., Peyre M. Extracapsular dissection in peripheral nerve schwannoma surgery using bright light and fluorescein sodium visualization: case series. Acta Neurochir (Wien). 2019 Dec; 161(12): 2447–2452. Epub 2019 Nov 2. PMID: 31679103.

10. Mahajan V., Rao S., Gupta P., et al. Angiosarcoma developing in a vagal schwannoma: A rare case report. Head Neck Pathol. 2015 Sep; 9(3): 405–411. Epub 2014 Nov 5. PMID: 25371276.

11. Brown R.W., Tornos C., Evans H.L. Angiosarcoma arising from malignant schwannoma in a patient with neurofibromatosis. Cancer. 1992 Sep 1; 70(5): 1141–1144. < 1141::aid-cncr2820700519>;2-q. PMID: 1515988.

12. Fuchs T.L., Nassour A.J., Glover A., et al. A proposed grading scheme for medullary thyroid carcinoma based on proliferative activity (ki-67 and mitotic count) and coagulative necrosis. Am J Surg Pathol. 2020 Oct; 44(10): 1419–1428. PMID: 32452872.

13. Nambiar S., Hegde V. Apoptosis in oral epithelial dysplastic lesions and oral squamous cell carcinoma: A prognostic marker. Indian J Pathol Microbiol. 2016 Jul-Sep; 59(3): 284–286. PMID: 27510661.

14. Prueter J., Norvell D., Backous D. Ki-67 index as a predictor of vestibular schwannoma regrowth or recurrence. J Laryngol Otol. 2019 Mar; 133(3): 205–207. PMID: 30983565.

15. Zelger B.G., Steiner H., Kutzner H., et al. Verocay body – prominent cutaneous schwannoma. Am J Dermatopathol. 1997 Jun; 19(3): 242–249. PMID: 9185909.

16. Akhtar M., Haider A., Rashid S., Al-Nabet A.D.M.H. Paget’s “seed and soil” theory of cancer metastasis: An idea whose time has come. Adv Anat Pathol. 2019; Jan; 26(1): 69–74. PMID: 30339548.

17. Amelot A., Terrier L.M., Mazeron J.J, et al. Timeline metastatic progression: in the wake of the “seed and soil” theory. Med Oncol. 2017 Oct 6; 34(11): 185. PMID: 28986775.

18. Eble J.A., Niland S. The extracellular matrix in tumor progression and metastasis. Clin Exp Metastasis. 2019 Jun; 36(3): 171–198. Epub 2019 Apr 11. PMID: 30972526.

19. Ishii G., Ochiai A., Neri S. Phenotypic and functional heterogeneity of cancer-associated fibroblast within the tumor microenvironment. Adv Drug Deliv Rev. 2016 Apr 1; 99(Pt B): 186–196. Epub 2015 Aug 14. PMID: 26278673.

20. Kuzet S.E., Gaggioli C. Fibroblast activation in cancer: when seed fertilizes soil. Cell Tissue Res. 2016 Sep; 365(3): 607–619. Epub 2016 Jul 29. PMID: 27474009.

21. Liu Q., Zhang H., Jiang X., et al. Factors involved in cancer metastasis: a better understanding to “seed and soil” hypothesis. Mol Cancer. 2017 Dec 2; 16(1): 176. PMID: 29197379.

Supplementary files

1. STROBE Statement—Authors Checklist .pdf
Type Research Instrument
Download (172KB)    
Indexing metadata


Views: 730

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.

ISSN 2218-7332 (Print)
ISSN 2658-3348 (Online)