Hybrid peripheral nerve sheath tumors

Yıl 2019, Cilt: 44 Sayı: 3, 804 - 810, 30.09.2019

Emine Kılıç Bağır Arbil Açıkalın Gülfiliz Gönlüşen Suzan Zorludemir Mehmet Ali Deveci

https://doi.org/10.17826/cumj.491823

Cited By: 2

Öz

Purpose: The aim of this study was to evaluate patients formerly diagnosed as neurofibroma and schwannoma in terms of hybrid peripheral nerve sheath tumors (PNSTs) via histopathological and immunohistochemical analysis. 

Materials and Methods: In this retrospective study, 115 patients formerly diagnosed as either neurofibroma or schwannoma were re-evaluated histopathologically. Among these patients, 32 cases which showed mixed morphology, suspicious for hybrid PNST were included in the study. Immunohistochemically, S100, CD34, EMA and ki67 were performed to these 32 cases, suspicious for hybrid PNST.

Results: Based on histopathology, 32 of 115 (27.8%) cases were suspicious for hybrid PNST. By the addition of immunohistochemical staining results; 22 of 32 cases were definitely diagnosed as hybrid PNST; of which 18 (81.8%) as schwannoma-neurofibroma and, 4 (18.2%) cases as schwannoma-perineurioma. 

Conclusion: Hybrid PNSTs are usually benign and have distinct histopathologic and immunohistochemistry findings. In the literature, rare case reports have described local recurrence and malignant transformation in hybrid PNSTs.  Therefore, further studies are needed to demonstrate the pathogenetic and prognostic significance of these tumors. Because of the risk of recurrence and malignancy potential, these tumors should be kept in mind in diagnosis of peripheral nerve sheath tumors.

Anahtar Kelimeler

Peripheral nerve sheath tumors, hybrid, immunohistochemistry, schwannoma, neurofibroma, perineuroma

Hibrid periferik sinir kılıfı tümörleri

Öz

Amaç: Nörofibrom ve schwannom tanısı alan olgularımızı histopatolojik ve immünohistokimyasal olarak hibrid periferik sinir kılıfı tümörü (PSKT) açısından değerlendirmektir.

Gereç ve Yöntem: Bu retrospektif çalışmada, daha önce nörofibrom ve schwannom tanısı almış 115 olgu histopatolojik bulguları ile tekrar değerlendirildi ve mikst morfolojiye sahip, hibrid PSKT şüphelenilen 32 hasta çalışmaya dahil edildi. Hibrid PNST şüphesi olan bu olgulara immünohistokimyasal olarak S100, CD34, EMA ve ki67 uygulandı.

Bulgular: Çalışmaya dahil edilen 115 hastanın histopatolojik olarak tekrar değerlendirmesinde; hibrid PSKT olduğu düşünülen 32(%27.8) hastanın 22 (%19.1)’si immünohistokimyasal veriler ile birlikte; 18 (%81.8)’i Schwannom-nörofibrom, 4’ü (%18.2) schwannom-perinörom olmak üzere “hibrit PSKT” tanısı aldı.

Sonuç: Hibrid PSKT' leri genellikle iyi huylu olup farklı histopatolojik ve immünhistokimyasal bulgulara sahiplerdir. Literatürde, hibrid PSKT' lerinin lokal rekürrens ve malign transformasyonu nadir olgu sunumları şeklinde bildirilmiştir. Bu tümörlerin patogenetik ve prognostik önemini göstermek için daha ileri çalışmalara ihtiyaç vardır. Bu tümörler, düşük de olsa rekürrens riski ve malignite potansiyelleri taşımaları nedeniyle  periferik sinir kılıfı tümörü tanısı verirken mutlaka akılda tutulmalıdır.

Anahtar Kelimeler

Periferik sinir kılıfı tümörleri, hibrid, immünohistokimya, schwannom, nörofibrom, perinöroma

Kaynakça

• 1. Rodrı´guez FJ, Folpe AL, Giannini C, et al. Pathology of peripheral nerve sheath tumors: diagnostic overview and update on selected diagnostic problems. Acta Neuropathol 2012;123:295–319.
• 2. Luca-Johnson J, Kalof AN. Peripheral nerve sheath tumors: an update and review of diagnostic challenges. Diagnostic Histopathology 2016; 22(11): 447-457
• 3. Soria-Céspedes D, Robles-Vidal C, Gómez-González A, Peñaloza-Ramírez R, Ortiz-Hidalgo C. Primary pleural hybrid cellular schwannoma/perineurioma: a case report. Respir Investig. 2014 Jul;52(4):269-73.
• 4. Kazakov DV, Pitha J, Sima R, Vanecek T et al. Hybrid peripheral nerve sheath tumors: Schwannoma-perineurioma and neurofibroma-perineurioma. A report of three cases in extradigital locations. Ann Diagn Pathol. 2005 Feb;9(1):16-23.
• 5. Michal M, Kazakov DV, Michal M. Hybrid peripheral nerve sheath tumors: A review. Cesk Patol. 2017 Spring;53(2):81-88.
• 6. Ud Din N, Ahmad Z, Abdul-Ghafar J, Ahmed R. Hybrid peripheral nerve sheath tumors: report of five cases and detailed review of literature. BMC Cancer. 2017 May 19;17(1):349.
• 7. Hornick JL, Bundock EA, Fletcher CD. Hybrid schwannoma/perineurioma: clinicopathologic analysis of 42 distinctive benign nerve sheath tumors. Am J Surg Pathol. 2009;33:1554–1561.
• 8. Ohata C, Imai N, Hinogami H, et al. Hybrid schwannoma/perineurioma: a report of two cases including a possible radiation-induced case. J Cutan Pathol. 2012;39:56–62.
• 9. Shelekhova KV, Danilova AB, Michal M, et al. Hybrid neurofibromaperineurioma: an additional example of an extradigital tumor. Ann Diagn Pathol. 2008;12:233–234.
• 10. Lang SS, ZagerEL, CoyneTM, et al. Hybrid peripheral nerve sheath tumor. J Neurosurg 2012;117:897–901.
• 11. Yang X, ZengY, Wang J. Hybridschwannoma/perineurioma: reportof 10 cases supporting a distinctiv entity.Int J Surg Pathol 2013;21:22–8.
• 12. Feany MB, Anthony DC, Fletcher CD. Nerve sheath tumours with hybrid features of neurofibroma and schwannoma: a conceptual challenge. Histopathology 1998;32:405–10.
• 13. Tsang WY, Chan JK, Chow LT, et al. Perineurioma: an uncommon soft tissue neoplasm distinct from localized hypertrophic neuropathy and neurofibroma. Am J Surg Pathol 1992;16:756 – 63.
• 14. Zelger B, Weinlich G. Perineuroma. A frequently unrecognized entity with emphasis on a plexiform variant. Adv Clin Path 2000;4:25-33.
• 15. Antonescu CR, Stemmer-Rachamimov AO, Perry A. Hybrid nerve sheath tumors. In: Louis DN, Ohgaki H, Wiestler OD, Cavenec WK, Ellison DW, et al., editors. WHO Classification of Tumors of the Central Nervous System Revised. 4th ed. IARC: Lyon; 2016. p. 224–5.
• 16. Harder A, Wesemann M, Hagel C, et al. Hybrid neurofibroma/schwannoma is overrepresented among schwannomatosis and neurofibromatosis patients. Am J Surg Pathol. 2012;36:702–9.
• 17. McLaughlin CT, Kaffenberger BH, Gru AA. A hybrid tumor with schwannoma-perineurioma-neurofibroma morphology. J Cutan Pathol. 2015 Nov;42(11):911-3.
• 18. Panda KM, Reena N. Intraneural Hybrid Neurofibroma/Schwannoma In Scalp: A Case Report. J Clin Diagn Res. 2015 Oct;9(10):ED05-6.
• 19. Hornick JL, Fletcher CD. Soft tissue perineurioma: clinicopathologic analysis of 81 cases including those with atypical histologic features. Am J Surg Pathol 2005; 29(7): 845-858.
• 20. Park JY, Park NJ, Kim SP, et al. A soft tissue perineuriona and hybrid tumor of perineurioma and schwannoma. Korean J Pathol 2012;46:75–8.
• 21. Erlandson RA. The enigmatic perineurial cell and its participation in tumors and in tumor like entities. Ultrastrc Pathol 1991;15:335–51.
• 22. Erlandson RA, Woodruff JM. Peripheral nerve sheath tumors: an electron microscopic study of 43 cases. Cancer 1982; 49(1): 273-287.
• 23. Hirose T, Sano T, Hizawa K. Ultrastructural localization of S-100 protein in neurofibroma. Acta Neuropathol 1986; 69(1-2): 103-110.
• 24. Zámečník M, Michal M. Perineurial cell differentiation in neurofibromas. Report of eight cases including a case with composite perineurioma- neurofibroma features. Pathol Res Pract 2001; 197(8): 537-544.
• 25. Hirose T, Tani T, Shimada T, et al. Immunohistochemical demonstration of EMA/Glut1-positive perineurial cells and CD34-positive fibroblastic cells in peripheral nerve sheath tumors. Mod Pathol 2003; 16(4): 293-298.
• 26. Michal M, Kazakov DV, Hadravský L, et al. Multivacuolated mucin-filled cells: a unique cell characteristic of plexiform neurofibroma. A report of 11 cases. Hum Pathol 2017; 60(2): 167-173.
• 27. Kacerovska D, Michal M, Kuroda N, et al. Hybrid peripheral nerve sheath tumors, including a malignant variant in type 1 neurofibromatosis. Am J Dermatopathol. 2013 Aug;35(6):641-9.
• 28. Ariza A, Bilbao JM, Rosai J. Immunohistochemical detection of epithelial membrane antigen in normal perineurial cells and perineurioma. Am J Surg Pathol 1988;12:678 - 83.
• 29. Theaker JM, Gatter KC, Puddle J. Epithelial membrane antigen expression by the perineurium of peripheral nerve and in peripheral nerve tumours. Histopathology 1988;13:171- 9.
• 30. Taubenslag KJ, Nickols HH, Chelnis JG, Mawn LA. Hybrid Neurofibroma/ Schwannoma of the supraorbital nerve: clinicopathologic correlation of a rare tumor. Ophthal Plast Reconstr Surg 2015;S104-S106.
• 31. Rekhi B, Jambhekar NA. Malignant transformation in a hybrid schwannoma/ perineurioma: Addition to the spectrum of a malignant peripheral nerve sheath tumour. Indian J Pathol Microbiol. 2011;54:825-28.
• 32. Baser ME, Friedman JM, Evans DG: Increasing the specificity of diagnostic criteria for schwannomatosis. Neurology 66:730–732, 2006