摘要
孤立性纤维性肿瘤(solitary fibrous tumor,SFT)是一种罕见的间叶源性肿瘤.虽然其大多数是良性,但去分化SFT在一些更为罕见的患者中也可能发生,该类肿瘤恶性程度更高,临床预后更差.本文报告1例腹腔去分化SFT患者,女,50岁,以"阵发性心悸、面部潮热半年"入住海南省人民医院,实验室检查均正常;下腹部CT显示:左侧肾上腺区有巨大分叶状软组织团块影,大小约为11.7 cm×10.7 cm,增强扫描示肿块可见明显不均匀强化.患者于泌尿外科接受手术治疗切除肿物.镜下见肿瘤由两部分组成,分别为梭形细胞区和肉瘤样区,梭形细胞区为典型SFT,肉瘤样区为去分化SFT.免疫组织化学检测示两区域均表达白细胞分数簇34(cluster of differentiation 34,CD34)、信号转导及转录激活因子6(signal transducer and activator of transcription 6,STAT6).Sanger测序检测到NAB2-STAT6基因融合,最终证实了去分化SFT的诊断.
Abstract
Solitary fibrous tumor(SFT)is a rare mesenchymal tumor.Although most SFT are benign,dedifferentiated SFT can occur in some rare cases,which are more malignant and have a worse clinical prognosis.This paper reports a case of dedifferentiated SFT in the abdominal cavity in a 50-year-old female patient,who was admitted to the Hainan General Hospital with paroxysmal palpitations and facial flushing for 6 months.Laboratory tests were all normal.Abdominal CT showed a large lobulated soft tissue mass in the left adrenal area,approximately 11.7 cm×10.7 cm in size,with significant heterogeneous enhancement on contrast-enhanced scan.The patient underwent surgical resection of the tumor in the urology department.Microscopically,the tumor consisted of two parts,a spindle cell area and a sarcomatous area.The spindle cell area was a typical SFT,and the sarcomatous area was a dedifferentiated SFT.Immunohistochemically testing showed that both areas expressed cluster of differentiation 34(CD34)and signal transducer and activator of transcription 6(STAT6).NAB2-STAT6 gene fusion was also detected,ultimately confirming the diagnosis of dedifferentiated SFT.
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