Kim Nah Ihm, Lee Ji Shin, Choi Chan, Nam Jong Hee, Choi Yoo Duk, Kim Hee Joon, Kim Sung Sun
Department of Pathology, Chonnam National University Hospital, Gwangju 61469, South Korea.
Department of Pathology, Chonnam National University Hwasun Hospital, Hwasun 58128, South Korea.
World J Clin Cases. 2022 Feb 26;10(6):2007-2014. doi: 10.12998/wjcc.v10.i6.2007.
Dedifferentiated liposarcoma (DDLPS) is an extremely rare neoplasm that exhibits various morphologies. The tumor is characterized by immunoreactivity to MDM2 and CDK4 and can be confirmed by detecting amplification fluorescence hybridization (FISH). Herein, we report an unusual case of DDLPS arising from the duodenum.
A 64-year-old man presented with repeated abdominal pain and weight loss. Radiologic studies revealed a mass of the duodenum involving the pancreas. The patient was treated with pylorus-preserving pancreaticoduodenectomy. Histologically, the tumor showed a high-grade sarcoma. Immunohistochemistry demonstrated that the tumor cells were positive for MDM2 and CDK4 expression. amplification was detected FISH, leading to the final diagnosis of DDLPS. Following surgery, the patient was treated in the intensive care unit due to peritonitis, and died 60 d after surgery.
To the best of the authors' knowledge, this is the first case of primary duodenal DDLPS in Korea and the third case in the English-language literature. Care must be taken not to misdiagnose DDLPS as another high-grade tumor. Liposarcoma should be in the differential diagnosis list.
去分化脂肪肉瘤（DDLPS）是一种极其罕见的肿瘤，具有多种形态。该肿瘤的特征是对MDM2和CDK4呈免疫反应性，可通过检测荧光原位杂交（FISH）扩增来确诊。在此，我们报告一例起源于十二指肠的罕见DDLPS病例。
一名64岁男性因反复腹痛和体重减轻就诊。影像学检查显示十二指肠有一肿块累及胰腺。患者接受了保留幽门的胰十二指肠切除术。组织学检查显示肿瘤为高级别肉瘤。免疫组织化学显示肿瘤细胞MDM2和CDK4表达阳性。FISH检测到扩增，最终诊断为DDLPS。术后患者因腹膜炎入住重症监护病房，术后60天死亡。
据作者所知，这是韩国首例原发性十二指肠DDLPS病例，也是英文文献中的第三例。必须注意不要将DDLPS误诊为其他高级别肿瘤。脂肪肉瘤应列入鉴别诊断清单。
