Kinebuchi Yoshiaki, Noguchi Wataru, Igawa Yasuhiko, Nishizawa Osamu
Department of Urology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan.
Int J Clin Oncol. 2005 Oct;10(5):353-6. doi: 10.1007/s10147-005-0495-8.
A 25-year-old man was referred to our hospital with left flank pain, and computed tomography (CT) and magnetic resonance imaging (MRI) revealed large retroperitoneal masses. Physical examination revealed many café-au-lait spots and superficial neurofibromas, and a diagnosis of neurofibromatosis type 1 (von Recklinghausen's disease) was made. The tumor was resected, and the pathological diagnosis was malignant peripheral nerve sheath tumor (MPNST). Six months after the operation, lung metastases were detected. Surgical resection was incomplete, as there were too many lesions. He received four courses of chemotherapy with carboplatin and etoposide, and the metastatic lung lesions were markedly decreased. After chemotherapy, complete resection of the remaining lung lesions was performed, and there has been no recurrence to date.
一名25岁男性因左侧腰痛被转诊至我院，计算机断层扫描（CT）和磁共振成像（MRI）显示腹膜后有巨大肿块。体格检查发现多处咖啡牛奶斑和浅表神经纤维瘤，诊断为1型神经纤维瘤病（冯雷克林霍增氏病）。肿瘤被切除，病理诊断为恶性外周神经鞘瘤（MPNST）。术后6个月，发现肺部转移。由于病灶太多，手术切除不彻底。他接受了四个疗程的卡铂和依托泊苷化疗，肺部转移病灶明显减少。化疗后，对剩余的肺部病灶进行了完整切除，至今未复发。
