Emine KILIÇ BAĞIR, Arbil AÇIKALIN, Gülfiliz GÖNLÜŞEN, Suzan ZORLUDEMİR, Mehmet Ali DEVECİ

Hibrid periferik sinir kılıfı tümörleri

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

Hybrid peripheral nerve sheath tumors

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.

Keywords:

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

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