Nikumbh Dhiraj B, Suryawanshi K H, Dravid N V, Patil T B, Rokade C M
Associate Professor, Department of Pathology, JMF's ACPM Medical College and Hospital , Dhule, Maharashtra, India .
J Clin Diagn Res. 2013 Jun;7(6):1155-8. doi: 10.7860/JCDR/2013/5511.3082. Epub 2013 Jun 1.
Malignant Peripheral Nerve Sheath Tumours (MPNST) are rare spindle- cell sarcomas which are derived from the schwann cells or the pleuripotent cells of the neural crest. MPNSTs are usually located in the peripheral nerve trunk roots, extremities and in the head and neck region. These tumours may arise as sporadic variants or in patients with neurofibromatosis1 (NF1). The estimated incidence of MPNSTs in the patients with NF1is 2-5% as compared to a 0.001% incidence in the general population. Herein, we are reporting a case of a giant sporadic (NF1 independent) low grade MPNST of the left thigh in 65 year female patient, which had a good prognosis.
恶性外周神经鞘瘤（MPNST）是一种罕见的梭形细胞肉瘤，起源于雪旺细胞或神经嵴的多能细胞。MPNST通常位于外周神经干根部、四肢以及头颈部区域。这些肿瘤可能以散发性变体形式出现，也可能出现在患有神经纤维瘤病1型（NF1）的患者中。与普通人群0.001%的发病率相比，NF1患者中MPNST的估计发病率为2-5%。在此，我们报告一例65岁女性患者左大腿巨大散发性（不依赖NF1）低级别MPNST病例，该病例预后良好。
