Is There Any Prognostic Relationship Between Malignant Peripheral Nerve Sheath Tumors And NF Type 1?

INTRODUCTION: In this study, we aimed to research the clinical features of malignant peripheral nerve sheath tumors (MPNST) which are rare soft tissue sarcomas and their prognostic relationship with Neurofibromatosis type 1 (NF1).
METHODS: The clinical features, treatment modalities, clinical outcomes and treatment failures of 42 (26 males, 16 females) patients operated for MPNST between January 2002 and December 2015 were evaluated retrospectively. 16 patients (38%) (10 males, 6 females, mean age 40 ± 14) were associated with NF1. 26 patients (62%) (16 males, 10 females, mean age 38 ± 15) were treated and followed up with sporadic MPNST diagnosis. The mean follow-up period was 30 months (± 12) in the NF1-associated group and 42 months (± 15) in the sporadic MPNST group. The most common site was extremity (85%) and mean tumor size was 11.2 cm (range between 2 and 21 cms). All patients underwent wide resection after diagnosis.
RESULTS: There was no significant difference between the two groups in terms of tumor size, tumor location, lymph node involvement and distant metastasis. In all patients, macroscopic surgery margin was negative, while 5 patients (31%) in the NF1-associated group and 9 patients (34%) in the sporadic group the margin was reported as positive (R1). Recurrence was seen in 20 of 42 patients (47%). Recurrence was seen in 13 patients (81%) in the NF1-associated group and 7 patients (26%) in the sporadic group. Mortality rate increased by 11.4 times in patients with positive microscopic surgical margin.
DISCUSSION AND CONCLUSION: The prognosis of malignant peripheral nerve sheath tumors developing on NF1 background is worse than sporadic type. In addition, tumor size, tumor depth and positive surgical margin are indicators of poor prognosis. The most important factor affecting survival is the presence of NF1 and the positive surgical margin.

Malign Periferik Sinir Kýlýfý Tümörlerinin NF Tip 1 ile Prognostik Ýliþkisi Var mýdýr?

GÝRÝÞ ve AMAÇ: Bu çalýþmada nadir görülen yumuþak doku sarkomlarýndan olan malign periferik sinir kýlýfý tümörlerinin (MPSKT) klinik özelliklerini ve Nörofibromatozis tip 1 (NF1) ile prognostik iliþkisini araþtýrmayý amaçladýk.
YÖNTEM ve GEREÇLER: Ocak 2002 ve Aralýk 2015 tarihleri arasýnda MPSKT nedeniyle opere edilen 42 (26 E, 16 K) hastanýn klinik özellikleri, tedavi yöntemleri, klinik sonuçlarý ve tedavi baþarýsýzlýklarý retrospektif olarak deðerlendirildi.16 hasta (%38) (10 erkek, 6 kadýn, ortalama yaþ 40± 14) NF1 ile iliþkili bulundu. 26 hasta (%62) (16 erkek, 10 kadýn, ortalama yaþ 38± 15) sporadik MPSKT tanýsý ile takip ve tedavi edildi. Ortalama takip süresi NF1 ile iliþkili grupta 30 ay (± 12) ve sporadik MPSKT grubunda 42 ay (± 15) idi. En sýk yerleþim yeri ekstremite (% 85) ve ortalama tümör boyutu 11,2 cm (daðýlým 2-21cm) idi. Tüm hastalara taný sonrasý geniþ rezeksiyon uygulandý.
BULGULAR: Ýki grup arasýnda tümör boyutu,tümör yerleþimi ve lenf nodu tutulumu ve uzak organ metastazý açýsýndan anlamlý fark yoktu.Tüm hastalarda makroskopik cerrahi sýnýr negatif iken,NF1 ile iliþkili grupta 5 hastada(% 31) ve sporadik grupta 9 hastada(% 34) mikroskobik cerrahi sýnýr pozitif (R1) olarak rapor edildi. 42 hastanýn 20’sinde (% 47) nüks görüldü. NF1 iliþkili grupta 13 hastada (% 81), sporadik grupta 7 hastada(% 26) nüks görüldü. Mikroskobik cerrahi sýnýr pozitif olan hastalarda mortalitenin 11,4 kat artýðý gözlenmiþtir.
TARTIÞMA ve SONUÇ: NF1 zemininde geliþen malign periferik sinir kýlýfý tümörlerinde prognoz sporadik tipe göre daha kötüdür. Ayrýca tümörün boyutu, derinliði, pozitif cerrahi sýnýr kötü prognoz göstergeleridir. Saðkalýmý etkileyen en önemli faktör NF1 varlýðý ve pozitif cerrahi sýnýrdýr.