6例恶性孤立性纤维性肿瘤的临床病理特征

Clinicopathological features in 6 cases of malignant solitary fibrous tumor

回顾性分析6例恶性孤立性纤维性肿瘤(malignant solitar y f ibrous tumor,MSFT)的临床病理特征,其中男5例,女1例;年龄25~69(中位数42.17)岁,分别发生在前额、膝部、大腿、锁骨上及胸腹壁,肿瘤直径1.8~24.5 cm。组织学特点:瘤细胞丰富密集,其间可见绳索样、玻璃样变胶原纤维分隔,瘤细胞呈短梭形、梭形,异型性明显,核分裂易见(≥4/10 HPF),瘤细胞呈束状、漩涡状排列,部分区域可见血管外皮瘤样结构,局灶可见出血或肿瘤性坏死。肿瘤部分区域可见浸润性边缘。免疫组织化学特点:6例均弥漫表达波形蛋白(Vimentin),STAT6,CD34,Bcl-2和CD99,1例的孕激素受体(progesterone receptor,PR)局灶阳性,6例的Ki-67为2%~60%(+)。S100,AE1/AE3,EMA,CD117,SOX10,Desmin,平滑肌肌动蛋白(smooth muscle actin,SMA),雌激素受体(estrogen receptor,ER)均阴性。MSFT的确诊主要依赖组织学的形态特征和免疫组织化学染色。形态学非常温和良善的SFT也可能有侵袭性生物学行为,临床上应尽早完整切除肿物并对孤立性纤维性肿瘤(solitar y fibrous tumor,SFT)长期随访,注意恶变的可能性。手术切除的完整性以及必要的放/化疗、靶向药物治疗等对预后非常重要。

The clinicopathological characteristics in 6 cases of malignant solitary fibrous tumor(MSFT) were retrospectively analyzed. Five cases were male, 1 case was female, aged 25 to 69 years old. The neoplasms located in forehead, knee, thigh, supraclavicular and thoracoabdominal wall, measured 1.8–24.5 cm in size. Histologically the tumor was composed of abundant and dense cells, which were separated by rope like collagenous fibrous stroma. The tumor cells were spindle to short-spindle shaped with marked nuclear atypia and increased mitotic activity. The tumor cells arranged in fascicular or swirl pattern. In focal areas, hemangiopericytoma-like structure, coagulative necrosis, focal hemorrhage and invasive margin were presented. Immunohistochemically, the tumor cells were diffusely positive for vimentin, STAT6, CD34, Bcl-2 and CD99;1 case focally positive for progesterone receptor(PR), all of them were negative for S100, AE1/AE3, EMA, CD117, SOX10, Desmin, smooth muscle actin(SMA) and estrogen receptor(ER), Ki-67 labelling index was 2%–60%. Diagnosis of MSFT mainly relied on its pathological morphology and immunohistochemical profiles. Th ere may be invasive biological behavior in SFT cases with moderate morphology, the tumor should be resected completely as soon as possible with long-term follow-up, beware of malignant transformation. Complete resection of the tumor combined with necessary chemotherapy, radiotherapy or targeted drugs were mostly important to the prognosis of malignant solitary fibrous tumor.