腺泡状软组织肉瘤的磁共振成像表现及临床病理学分析

Magnetic resonance imaging findings and clinicopathologic features of alveolar soft-part sarcoma

目的:分析腺泡状软组织肉瘤(alveolar soft-part sarcoma,ASPS)的磁共振成像(magnetic resonance imaging,MRI)表现和病理学特征。方法:选取2011年-2018年经手术后病理学检查确诊的ASPS患者6例,患者均接受MRI检查,对其临床表现、影像学及病理学特点进行综合分析。结果:患者ASPS发生于耳前1例,舌部2例,四肢3例。肿块呈圆形或类圆形,T1WI呈等或稍高信号;T2WI呈不均匀高信号,肿瘤内部及周围可见粗大、迂曲的流空血管影。扩散加权成像(diffusion-weighted imaging,DWI)呈不均匀高信号,增强后出现不均匀强化。病理学检查发现肿瘤细胞呈多角形,细胞核圆,有明显的核仁,胞质丰富,呈器官状或腺泡状排列。免疫组织化学染色阳性率较高的标志物为TFE3(3/3)、NSE(4/6)和MyoD1(3/6)。结论:ASPS具有特征性MRI表现和临床特点,年轻女性多见,好发于四肢深部软组织,体积较大,T1WI呈等或稍高信号,T2WI显示肿瘤内部及周围可见粗大、迂曲的流空血管影。预后较差,极易发生转移。

Objective:To investigate the magnetic resonance imaging(MRI)manifestations,clinical and pathological features of alveolar soft-part sarcoma(ASPS).Methods:The clinical data of 6 patients with ASPS confirmed by surgery and pathology from 2011 to 2018 were retrospectively analyzed,and all patients underwent MRI examination.Its clinical manifestations,imaging and pathological features were comprehensively analyzed.Results:Among 6 patients,1 case occurred in front of ear,2 cases in tongue and 3 cases in limbs.The mass was round or nearly round,and the T1 WI was equisignal or slightly high signal.The T2 WI was non-uniform high signal;large and tortuous empty vascular shadows were seen in and around the tumorthe dispersion was high signal.Diffusion-weighted imaging(DWI)showed high signal unevenness,and the enhancement was non-uniform enhancement.Microscopically,the tumor cells were polygonal,with round nuclei,visible nucleoli and abundant cytoplasm.The positive rate of immunohistochemistry was higher:TFE3(3/3),NSE(4/6),MyoD1(3/6).Conclusion:ASPS has characteristic MRI manifestations and clinical characteristics,which are more common in young women and tend to occur in soft tissues deep in the limbs,with large volume,equal or slightly high signal on T1 WI,and large,tortuous empty vascular shadow in and around the tumor on T2 WI.The prognosis is poor and metastasis is very likely to occur.