胰腺原发性去分化脂肪肉瘤:案例报道和文献综述

Primary dedifferentiated liposarcoma of the pancreas:A case report and literature review

背景去分化脂肪肉瘤(dedifferentiated liposarcoma,DDLPS)是一种高级别侵袭性疾病,好发于腹膜后,其次为大腿和腹股沟处,而发生在胰腺的DDLPS非常罕见.病例简介本例患者因上腹部隐痛而行CT检查提示胰腺占位性病变,性质不明.根据患者术前病史、查体、辅助检查,考虑胰腺头颈部巨大占位性病变,边界尚清,存在手术指征,于我院接受胰十二指肠切除术,术后病理提示胰腺DDLPS,术后3 mo患者复查提示肿瘤复发,予达卡巴嗪联合表柔比星化学治疗后效果不佳,18 mo后去世.结论由于胰腺DDLPS通常对放化疗不明显,因此,目前治疗胰腺DDLPS最有效的治疗方法为显微镜下的阴性切除.但由于发生于胰腺的脂肪肉瘤位置较深,且临近周围许多器官,这使得肿瘤很难完全被切除,许多患者因此会发展为区域性复发性疾病.

BACKGROUND Dedifferentiated liposarcoma(DDLPS)is a high-grade invasive tumor that more commonly occurs in the retroperitoneum,followed by the thighs and groin.DDLPS in the pancreas is very rare.CASE SUMMARY A patient underwent CT examination due to hidden pain in the upper abdomen,which revealed a pancreatic space occupying lesion of unknown nature.Based on the patient’s preoperative medical history,physical examination,and auxiliary examination,it was considered that there was a huge space occupying lesion in the head and neck of the pancreas,with clear boundaries and surgical indications.The patient underwent pancreaticoduodenectomy at our hospital,and the postoperative pathology revealed a DDLPS of the pancreas.Three months after the surgery,tumor recurrence occurred.Dacarbazine combined with epirubicin chemotherapy had a poor effect,and the patient passed away 18 mo later.CONCLUSION Due to the fact that DDLPS of the pancreas is usually not significantly responsive to radiotherapy and chemotherapy,the currently most effective treatment method for DDLPS of the pancreas is resection with negative margin under a microscope.However,due to the deep location of liposarcoma in the pancreas and its proximity to many surrounding organs,it is difficult to completely remove the tumor,and many patients may develop regional recurrence.