Hoshimoto Sojun, Morise Zenichi, Takeura Chinatsu, Ikeda Masahiro, Kagawa Tadashi, Tanahashi Yoshinao, Okabe Yasuhiro, Mizoguchi Yoshikazu, Sugioka Atsushi
Department of Surgery and.
Rare Tumors. 2009 Dec 28;1(2):e27. doi: 10.4081/rt.2009.e27.
We report an extremely rare case of malignant Triton tumor developing in the retroperitoneal space in a patient with neurofibromatosis type 1. A 21-year old man who had been diagnosed with neurofibromatosis type 1 was admitted to our hospital with the chief complaint of a palpable abdominal mass. Abdominal computed tomography revealed a huge heterogeneous tumor measuring approximately 17 cm in diameter occupying the left retroperitoneal space, and numerous metastatic lesions between the left psoas muscle and the left thigh with dissolution of the left hip joint. After the diagnosis of a retroperitoneal malignant neurogenic tumor, resection of the tumor with reconstruction of the abdominal aorta was conducted, followed by postoperative transarterial infusion chemotherapy. The histopathological diagnosis was malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation, namely malignant Triton tumor. Postoperative chemotherapy was in vain and the patient died 14 months after the surgery as a result of lung metastasis.
我们报告了1例极其罕见的恶性蝾螈瘤，发生于1型神经纤维瘤病患者的腹膜后间隙。一名21岁男性，此前已被诊断为1型神经纤维瘤病，因可触及腹部肿块为主诉入院。腹部计算机断层扫描显示一个巨大的异质性肿瘤，直径约17 cm，占据左腹膜后间隙，左腰大肌与左大腿之间有大量转移灶，左髋关节溶解。在诊断为腹膜后恶性神经源性肿瘤后，进行了肿瘤切除及腹主动脉重建，随后进行术后经动脉灌注化疗。组织病理学诊断为伴有横纹肌肉瘤分化的恶性周围神经鞘瘤，即恶性蝾螈瘤。术后化疗无效，患者术后14个月因肺转移死亡。
