Türk Nöroşirürji Dergisi
2013 , Vol 23 , Num 3
Malignant Peripheral Nerve Sheath Tumor Developing on the Background of a Neurofibroma Originating from a Cervical Nerve Root in Neurofibromatosis Type 1: A Case Report
1Dokuz Eylül Üniversitesi, Tıp Fakültesi, Nöroşirürji Anabilim Dalı, İzmir, Türkiye2İzmir Bozyaka Eğitim ve Araştırma Hastanesi, Nöroşirürji Kliniği, İzmir, Türkiye
3İzmir Bozyaka Eğitim ve Araştırma Hastanesi, Patoloji Kliniği, İzmir, Türkiye AIM: A malignant peripheral nerve sheath tumor (MPNST) develops in 2-29% of the patients with NF1.
CASE REPORT: A 33-year-old case with neurofibromatosis Type 1 (NF1) with MPNST originating from the left sixth cervical nerve root is presented. The case was diagnosed as MPNST developing on a background of neurofibroma with the clinical history of NF1, detection of neurofibromatous morphology, and the presence of relevant histopathological and immunohistochemical findings. The lesion was excised completely without any neurological deterioration using microsurgical methods, and the patient has been followed up clinically.
CONCLUSION: MPNSTs seen in association with NF1 have a poor prognosis because of the occurrence of metastases and recurrences.
Anahtar Kelimeler : Malignant peripheral nerve sheath tumor, Neurofibromatosis Type 1, Neurofibroma