Sato Katsunari, Ashitomi Yuya, Takahashi Ryosuke, Ashino Koki, Sugawara Shuichiro, Futakuchi Mitsuru, Motoi Fuyuhiko
First Dept. of Surgery, Yamagata University.
Gan To Kagaku Ryoho. 2022 Dec;49(13):1411-1413.
Dedifferentiated liposarcoma shows poor prognosis because of poor resectability due to aggressive invasion of adjacent organs with multicentric growth and its low sensitivity to chemotherapy. We report herein a case of a giant dedifferentiated liposarcoma, successfully treated by pancreaticoduodenectomy(PD)for tumor reduction and chemotherapy for 1 year after surgery, followed by additional surgery for tumor reduction. The patient is a woman in 50s. CT showed an 18.5×9 cm main mass surrounding the superior mesenteric artery(SMA and SMV)with multiple tumors in the pelvis. Needle biopsy revealed dedifferentiated liposarcoma. Although complete resection or chemotherapy was not feasible, surgery was performed for local control and introduction of chemotherapy. The main tumor was resected by PD with SMV resection and right colectomy. Chemotherapy with doxorubicin followed by eribulin was administered after surgery. The residual lesions were controlled for 1 year. Partial resection of the tumors in the mesentery was performed. Eribulin were administered starting postoperatively. One year and 10 months after the initial surgery, there was no progress in residual disease. Although R2 resection for dedifferentiated liposarcoma shows extremely poor prognosis. Even when complete resection would be difficult, multidisciplinary treatment including debulking surgery might be effective for disease control.
去分化脂肪肉瘤预后较差，原因在于其多中心生长并侵袭邻近器官，导致手术切除困难，且对化疗敏感性低。本文报告一例巨大去分化脂肪肉瘤患者，通过胰十二指肠切除术（PD）缩小肿瘤，并在术后进行1年化疗，随后再次手术进一步缩小肿瘤。患者为一名50多岁的女性。CT显示一个18.5×9 cm的主要肿块围绕肠系膜上动脉（SMA和SMV），盆腔内有多个肿瘤。穿刺活检显示为去分化脂肪肉瘤。尽管无法进行完整切除或化疗，但为了局部控制和引入化疗还是进行了手术。通过PD切除主要肿瘤，并切除SMV和右半结肠。术后给予阿霉素化疗，随后使用艾瑞布林。残余肿瘤得到控制达1年。对肠系膜肿瘤进行了部分切除。术后开始使用艾瑞布林。初次手术后1年零10个月，残余疾病无进展。尽管去分化脂肪肉瘤的R2切除预后极差。即使完整切除困难，包括减瘤手术在内的多学科治疗可能对控制疾病有效。
