腹膜后恶性孤立性纤维性肿瘤的临床病理分析

Clinicopathological analysis of retroperitoneal malignant solitary fibrous tumor

目的探讨腹膜后恶性孤立性纤维性肿瘤(MSFT)的临床病理特征、病理诊断、治疗和预后。方法收集2002年1月至2018年2月确诊的7例MSFT患者的临床病理资料,对其临床表现、组织病理学特征、免疫表型、治疗及预后进行分析。结果 7例患者中男性4例,女性3例;中位年龄51岁(范围:22~73岁)。所有患者均接受根治性手术切除。2例患者术后接受异环磷酰胺联合表柔比星方案化疗6个周期,1例接受顺铂联合丝裂霉素术中腹腔热灌注化疗。随访截止于2019年1月,1例无瘤生存,2例死亡,4例带瘤生存;4例复发2次,1例复发4次。MSFT病理特征为肿瘤界限清楚,可有包膜或者假包膜,肿瘤边缘浸润性生长,可见"鹿角状"血管,有坏死和出血,肿瘤细胞丰富,细胞呈中度至重度异型性,核分裂象多见(≥2个/10HPF)。CD34、Bcl-2、STAT6、CD99和Vimentin蛋白均呈阳性表达。结论腹膜后MSFT确诊主要依赖病理形态学和免疫表型。MSFT术后易复发,需长期随访。

Objective To explore the clinical and pathological features,pathological diagnosis,treatment and prognosis of retroperitoneal malignant solitary fibrous tumor(MSFT).Methods Seven cases of retroperitoneal MSFT were reviewed from January 2002 to February 2018.Clinicopathological features,immunohistochemical staining,treatment and prognosis were analyzed among the 7 patients.Results Of the 7 patients,4 were male and 3 were female.The median age was 51 years(22-73 years).All the 7 cases received surgical resection as the first treatment,and 2 cases had ifosfamide and doxorubicin chemotherapy for 6 cycles and 1 case underwent intraoperative hypertermic intraperitoneal chemotherapy with cisplatin and mitomycin.Until January 2019,there were 1 case alive without disease,2 cases of death,and 4 cases alive with disease.Four cases recurred twice,and 1 case recurred 4 times.Pathologically,MSFT usually had clear psuedocapsule with invasion to the capsule.The histopathology shows abundant tumor cells with obvious heteromorphosis,significant atypia,and prominent pathological mitosis(≥2/10HPF).“Deer horn like”blood vessels,hemorrhage and necrosis were also evident.The protein expression of CD34,Bcl-2,STAT6,CD99 and Vimentin were positive.Conclusion Definite diagnosis of MSFT is mainly based on its pathological morphology and immunohistochemical results.MSFT is easy to recur and needs long-term follow-up.