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Year : 2015  |  Volume : 1  |  Issue : 1  |  Page : 45-48

Malignant peripheral nerve sheath tumor

1 Department of Surgery, Jersey City Medical Center-Barnabas Health, Jersey City, New Jersey, USA
2 Department of Surgery, St. Luke's University Health Network, Bethlehem, Pennsylvania, USA
3 Department of Anesthesiology, The Ohio State University College of Medicine, Columbus, Ohio, USA

Correspondence Address:
Stanislaw P Stawicki
Department of Surgery, St. Luke's University Health Network, 801 Ostrum Street, Bethlehem, Pennsylvania
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Source of Support: None, Conflict of Interest: None

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Type 1 neurofibromatosis (NF1) is also known as von Recklingausen's disease. Among patients affected by NF1, fast-growing plexiform type neurofibromas have a high rate of malignant transformation and need to be distinguished from benign plexiform neurofibromas. Here, we describe an emergent case presentation with rapid transformation of neurofibromatosis into a malignant peripheral nerve sheath tumor in a male patient in his early 20's, who was admitted with a 4-day history of urinary retention, pelvic “fullness,” and lower back pain. Highlighted are computed tomography imaging characteristics of malignant peripheral nerve sheath tumor, exemplified by a large retroperitoneal/pelvic mass compressing the bladder and both ureters, resulting in bilateral hydronephrosis and urinary retention, and prompting urgent surgical resection. Associated MRI images and an intraoperative photograph are also presented. The following core competencies are addressed in this article: Patient care, Medical knowledge, Systems-based practice

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