成人腺泡状软组织肉瘤的影像学特征

Imaging features of adult alveolar soft part sarcoma

目的:探讨成人腺泡状软组织肉瘤(alveolar soft part sarcoma,ASPS)的CT、MRI及PET/CT影像学特征。方法:回顾性分析2012年1月—2016年12月经手术或空心针活检病理证实的10例ASPS病例,分析其临床表现及CT、MRI、PET/CT影像学特征。结果:10例患者中位年龄29岁(22~46岁),发生于四肢7例(其中大腿4例),盆腔3例;中位大小72 mm(29~260 mm)。行MRI检查的6例病灶T1WI均呈高信号,T2WI呈不均匀高信号4例,高低信号混杂2例;CT及MRI增强后所有病灶均呈明显不均匀强化,且于病灶周围或病灶内见增粗增多的血管影。PET/CT示所有原发灶及绝大多数转移灶氟脱氧葡萄糖(fluorodeoxyglucose,FDG)明显摄取。有9例患者发生转移,其中肺部转移9例,淋巴结转移3例,骨转移2例,皮下及脑部转移各1例。结论:成人ASPS发病年龄较小,好发于四肢。MRI平扫T1WI呈高信号、CT或MRI增强后肿瘤周围及瘤内明显增粗增多的血管影及明显不均匀强化为其影像学特征。肺部转移极为常见。

Objective： To explore the CT, MRI and PET/CT imaging features of adult alveolar soft part sarcoma （ASPS）. Methods： The clinical data, CT, MRI and PET/CT imaging features of ten patients with histopathologically proved ASPS from January 2012 to December 2016 were retrospectively reviewed. Results： The median age of the ten patients was 29 years （22-46 years）. The median size of the lesions was 72 mm （29-260 mm）. Seven lesions were in the extremities （4 cases in the thigh）, and 3 cases in the pelvic. Tumors were hyper-intensity on TIWI and heterogeneous hyper-intensity or mixed hypo-/hyper-intensity on T2WI. All lesions showed marked heterogeneous enhancement and numerous large vessels on both contrast-enhanced T1WI and contrast-enhanced CT. All primary lesions and most of metastases were fluorodeoxyglucose （FDG）-avid on PET/CT. And metastases developed in 9 cases （9 cases in lungs, 3 cases in lymph nodes, 2 cases in bones, 1 case in subcutaneous tissues and 1 case in brain）. Conclusion： Adult ASPS predominantly develops in the extremities of young patients. Tumors show hyper-intensity on T1WI, and marked heterogeneous enhancement and numerous large vessels on both contrast-enhanced T1WI and contrast-enhanced CT. Pulmonary metastasis develops in the majority of ASPS patients.