Miura Tetsuya, Yamada Yuji, Muramaki Mototsugu, Tanaka Kazushi, Hara Isao, Fujisawa Masato
The Division of Urology, Department of Organs Therapeutics, Faculty of Medicine, Kobe University Graduate School of Medicine.
Hinyokika Kiyo. 2006 Mar;52(3):207-9.
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.
我们报告了1例1型神经纤维瘤病患者发生的腹膜后恶性外周神经鞘瘤（MPNST）。一名42岁女性因可触及左腹部肿块入院。她的母亲、儿子和女儿都患有1型神经纤维瘤病。计算机断层扫描和磁共振成像显示一个73×76mm的腹膜后肿块。我们对肿瘤进行了完整切除，并在术中通过冰冻切片检查确认切缘情况。组织病理学检查显示为MPNST。尽管未进行进一步治疗，但术后11个月她仍存活，且无疾病证据。
