Rai Bansal Anand, Singh Griwan Mahavir, Rajan Karthikeyan Yayathi, Kumar Singh Santosh
Department of Surgery and Urology, Sharma Postgraduate Institute of Medical Sciences, Rohtak, India.
Nephrourol Mon. 2013 Jul 1;5(3):843-6. doi: 10.5812/numonthly.8029. Epub 2013 May 25.
Malignant peripheral nerve-sheath tumor (MPNST) is a high-grade malignant tumor of ecto-mesenchymal origin comprising approximately 10% of soft-tissue sarcomas. They tend to occur associated with neurofibromatosis or sporadically. Here we report a MPNST at an extremely rare location and behavior causing disappearance of the entire kidney. Patient evaluation included clinical, biochemical and radiological studies before treatment. Histological study revealed the rare diagnosis. Patient was asymptomatic at six-month follow-up after treatment.
恶性外周神经鞘瘤(MPNST)是一种起源于外胚层间叶组织的高级别恶性肿瘤,约占软组织肉瘤的10%。它们往往与神经纤维瘤病相关或散发性发生。在此,我们报告一例发生于极其罕见部位且导致整个肾脏消失的MPNST。患者评估包括治疗前的临床、生化和影像学检查。组织学研究得出了这一罕见诊断。患者在治疗后6个月的随访中无症状。