Ogawa Toshihiro, Harano Masao, Morito Toshiaki, Ichimura Kouichi, Choda Yasuhiro, Matsukawa Hiroyoshi, Ojima Yasutomo, Idani Hitoshi, Shiozaki Shigehiro, Okajima Masazumi
Dept. of Surgery, Hiroshima City Hiroshima Citizens Hospital.
Gan To Kagaku Ryoho. 2017 Nov;44(12):1787-1789.
A 60-year-old woman presented at our hospital with abdominal pain and vomiting.Three abdominal tumors were detected, and she was referred to our department for surgery.She underwent an enterectomy including 2 of the tumors.The third tumor could not be resected because it had invaded the superior mesenteric artery(SMA)and superior mesenteric vein(SMV). Based on positive immunohistochemistry results for S-100 protein and desmin, nerve sheath differentiation with rhabdomyoblastic differentiation was confirmed, and she was diagnosed with a malignant triton tumor(MTT).She received postoperative chemotherapy with adriamycin and dacarbazine.However, she died 7 months after surgery, with multiple peritoneal metastases.MTT is a subtype of malignant peripheral nerve sheath tumor and is very rare.MTT has a poor prognosis with a high tendency of local recurrence and early metastasis.The most common treatment strategy is radical resection followed by postoperative chemoradiotherapy.When preoperative diagnosis is possible, radical resection with clear margins should be performed.
一名60岁女性因腹痛和呕吐前来我院就诊。检查发现腹部有三个肿瘤，遂转诊至我科接受手术。她接受了肠切除术，切除了其中两个肿瘤。第三个肿瘤因侵犯肠系膜上动脉（SMA）和肠系膜上静脉（SMV）而无法切除。根据S-100蛋白和结蛋白免疫组化结果呈阳性，证实为伴有横纹肌母细胞分化的神经鞘分化，她被诊断为恶性蝾螈瘤（MTT）。她术后接受了阿霉素和达卡巴嗪化疗。然而，她术后7个月死于多处腹膜转移。MTT是恶性周围神经鞘瘤的一种亚型，非常罕见。MTT预后较差，局部复发和早期转移倾向高。最常见的治疗策略是根治性切除，术后进行放化疗。若能进行术前诊断，应行切缘清晰的根治性切除。
