title: [A case of retroperitoneal malignant peripheral nerve sheath tumor in a patient with neurofibromatosis].
abstract: We report a case of retroperitoneal malignant peripheral nerve sheath tumor (MPNST) in a patient with neurofibromatosis 1. A 42-year-old woman was admitted because of a palpable left abdominal mass. Her mother, son, and daughter had neurofibromatosis 1. Computed tomography and magnetic resonance imaging revealed a 73 x 76 mm retroperitoneal mass. We performed complete resection of the tumor, confirming the margin status by frozen section examination intraoperatively. The histopathological examination revealed MPNST. Although no further therapy was performed, she is alive with no evidence of disease 11 months after surgery.
authors.affiliation: The Division of Urology, Department of Organs Therapeutics, Faculty of Medicine, Kobe University Graduate School of Medicine.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Miura
authors.forename: Yuji
authors.initials: Y
authors.lastname: Yamada
authors.forename: Mototsugu
authors.initials: M
authors.lastname: Muramaki
authors.forename: Kazushi
authors.initials: K
authors.lastname: Tanaka
authors.forename: Isao
authors.initials: I
authors.lastname: Hara
authors.forename: Masato
authors.initials: M
authors.lastname: Fujisawa
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D009456:Neurofibromatosis 1 / Q000150:complications* / Q000235:genetics; D011379:Prognosis; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D014057:Tomography, X-Ray Computed