胰腺多形性未分化肉瘤:病例报告与文献回顾

Undifferentiated pleomorphic sarcoma of pancreas:case report and literature review

背景与目的:多形性未分化肉瘤(UPS),既往称为恶性纤维组织细胞瘤(MFH)是一种间叶源性恶性肿瘤,占所有软组织肉瘤的20%以上,多发生于四肢、躯干及腹膜后。发生于胰腺的UPS极为罕见。国内、外虽有报道,但多以个案形式报道,缺少大宗病例资料分析。本研究就笔者团队诊治的1例胰腺UPS患者,结合文献,探讨其临床特点、诊疗及预后。方法:回顾分析内蒙古医科大学附属医院肝胆外科诊治的1例胰腺UPS患者的临床资料,同时检索国内外数据库中的相关文献资料,将同类病例资料汇总分析。结果:共32例(笔者单位1例加文献报告病例31例)纳入研究,其中男20例,女12例;中位年龄54.6(22~74)岁。按病理类型分为:巨细胞型9例,黏液型5例,多形性型18例;按发生部位分为:胰头13例,胰体尾12例,胰体3例,胰尾2例,全胰腺2例。术前诊断为胰腺癌8例,胰腺肿物及占位6例,假性囊肿4例,囊腺瘤4例,胰腺肿瘤3例,间质肿瘤1例,纤维肉瘤1例,炎性病变1例,未提及术前诊断4例。临床表现大多表现为快速软组织肿块进行性增大,常伴有压迫症状,可伴有恶心呕吐,上腹部不适、腹胀,体质量减轻或黄疸。所有患者的术前诊断中无正确诊断。32例均行手术治疗,围术期死亡1例,总生存期15.3个月。结论:胰腺UPS为高度恶性肿瘤,无明显特异性临床表现,极易发生转移及复发,预后差。早期、完整切除肿瘤以获得R0切除是手术治疗的核心,但是由于胰腺UPS的特殊组织生物学行为,在部分病例中即使施行广泛切除或根治性切除,仍不能避免肿瘤局部复发或远处转移。术后给予放化疗有可能能够改善患者的生存。

Background and Aims:Undifferentiated pleomorphic sarcoma(UPS),previously known as malignant fibrous histiocytoma(MFH),is a malignant tumor of mesenchymal origin,accounting for more than 20%of all soft tissue sarcomas,most of which occur in the limbs,body trunk and retroperitoneal space.The occurrence of UPS in the pancreas is extremely rare.Although there are some reports at home and abroad,most of them are presented as individual case report,and the data analysis of larger series of patients are scarce.This article was written to discuss the clinical characteristics,diagnosis and treatment as well as the prognosis of this condition through analyzing one patient case of pancreatic UPS treated by the author’s team combined with the literature data.Methods:The clinical data of one patient with pancreatic UPS diagnosed and treated in the Department of Hepatobiliary Surgery of the Affiliated Hospital of Inner Mongolia Medical University was retrospectively analyzed.Meanwhile,a search of the relevant literature from the national and international databases was performed.Then,a pooled analysis of the same medical cases was conducted.Results:There a total of 32 patients(including our case and 31 cases reported in the literature)were enrolled in the study.Of the patients,20 cases were males and 12 case were females,with a median age of 54.6(22–74)years.The pathological types were classified as giant cell tumor in 9 cases,myxoid tumor in 5 cases,and pleomorphic tumor in 18 cases.The tumors were located in the head of the pancreas in 13 cases,in the body and tail of the pancreas in 12 cases,in the body of the pancreas in 3 cases,in the tail of the pancreas in 2 cases and in whole pancreas in 2 cases.Preoperative diagnosis was pancreatic cancer in 8 cases of,pancreatic tumor and space occupying lesion in 6 cases,pseudocyst in 4 cases,cystadenoma in 4 cases,pancreatic tumor in 3 cases of,and interstitial tumor,fibrosarcoma,inflammatory lesion in one case each and unavailable in 4 cases.The clinical manifestations included rapidly progressive enlargement of soft tissue lump,frequently accompanied with pressure symptoms or nausea and vomiting,epigastric discomfort,abdominal distension,weight loss or jaundice.The correct diagnosis was made in none of the patients.All the 32 patients underwent surgical treatment,perioperative death occurred in one case,and the overall survival time is 15.3 months.Conclusion:The pancreatic UPS is a highly malignant tumor with no specific clinical manifestations,high likelihood of metastasis and recurrence,and dismal prognosis.Early and complete resection of the tumor to obtain R0 resection is essential for surgical treatment.However,even if extensive resection or radical resection is performed in some cases,local tumor recurrence or distant metastasis still cannot be avoided,due to the special biohistological characteristics of pancreatic UPS.Postoperative radiotherapy and chemotherapy may probably improve survival of the patients.