ISSN: 1300-0292
İndekslendiği Dizinler: SCIENCE CITATION INDEX EXPANDED
CINAHL, Index Copernicus,
Chemical Abstracts (CA),
Excerpta Medica / EMBASE
Dil: Türkçe, İngilizce
İçerik: Orijinal Araştırma, Derleme, Editöre Mektup, Olgu Sunumu, Tıp Eğitimi, Tıbbi Kitap İncelemeleri
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Malignant Peripheral Nerve Sheath Tumorin A Case With Neurofibromatosis Type I
Mustafa GÖKÇE*, Tuba T.KIZILTEPE**
* Uz.Dr., Ankara Onkoloji Hastanesi Nöroloji Bölümü
**Uz.Dr., Ankara Onkoloji Hastanesi Radyoloji Bölümü, ANKARA
Purpose: The incidence of malign peripheral nerve sheath tumors (MPNST) in patients with Neurofibromatosis type 1 (NF1) has been estimated to be 2% to 5% compared with 0.0001%in the general population. The purpose of this paper is to present the difficulties of diagnosis and to discuss the clinical manifestations, imaging features and histopathological findings of a MPNST in a patient with NF1. Case Report: A 65 year old woman presented with one year history of progressive right radicular limb pain. Electromyography (EMG), magnetic resonance imaging (MRI), biopsy and computerized tomography (CT) was performed. EMG revealed positive sharp wave and fibrillation potentials at the right lomber paraspinal muscle. The MR images demonstrated a right paravertebral mass. Although the mass was unresectable at surgery, the biopsy revealed benign PNST. The pain increased progressively. A follow-up CT was revealed the rapid growth of the mass. Rapid clinical deterioration and the imaging features of the mass was evaluated again and the tumor was considered a malign PNST.Conclusion: If a patient has a progressively increasing radicular pain, one should bring into mind the possibility of a paravertebral mass due to neurofibromatosis.Recognition of imaging features suggesting malignancy is important for prospective diagnosis and to help to guide therapy in the clinical management of these patients.Keywords: Neurofibromatosis 1, Nerve Sheath Tumors, Magnetic Resonance Imaging, DiagnosisTurkiye Klinikleri J Med Sci 2003, 23:230-233
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