四肢远端型上皮样肉瘤的MRI表现与病理对照

MRI Findings of Distal Epithelioid Sarcoma in Extremities:Comparison with Pathology

目的探讨四肢远端型上皮样肉瘤(DES)的MRI表现与病理学基础。方法回顾性分析8例经病理证实的四肢DES患者的MRI及病理资料。结果 8例中,2例单发病变(长径≤5 cm)呈结节状等或长T_1、长T_2信号;1例单发病变(长径>5 cm)及5例多发病变呈混杂T_1、混杂T_2信号。5例出现多发囊变,2例囊变内出现短T_2液-液平面。3例出现小片状短T_1信号和/或环状短T_2信号(新鲜和/或陈旧出血)。2例病变沿长骨长轴呈"葡萄串"样分布。8例软组织均广泛受累,其中3例骨质受累,呈斑点或片状长T_1、长T_2信号。增强扫描8例病变均呈片状或花环状明显强化,受累的软组织及骨质轻度至明显强化。结论当四肢出现复发性软组织肿物,MRI发现病变有明显出血、沿着长骨长轴分布、多发囊变及囊内出血、骨质受累但结构破坏不明显等特点时,应当考虑到DES的可能,但确诊最终依赖病理。

Objective To explore the MRI findings and pathological features of distal-type epithelioid sarcoma（ DES）in the extremities. Methods The MRI images and pathological results of eight DES cases comfirmed by pathology were retrospectively studied. Results of 8 cases,2 cases with solitary lesions（ major diameter ≤5 cm） were shown as T1-isointensity / hypointensity,T2-hyperintensity nodules. 1 case with solitary lesion（ major diameter 5 cm） and 5 cases with multiple lesions were T1- and T2- heterogeneous intensity. 5 cases were shown as multiple cysts and 2 of which T2-hypointensity ＂fluid-fluid level＂could be found. Patchy or irregular T1-hyperintensity and / or T2-hypointensity areas（ fresh and /or remote hemorrhage） were found in 3 cases. The lesions had a distribution along the long bones like grapes in 2 cases.Adjacent soft tissues were widely invaded in all cases. Dotted or patchy T1-hypointensity and T2-hyperintensity areas could be found in 3 bone invasion cases. All lesions displayed patchy or ring-like obvious enhancement,while the affected soft tissue and bone displayed slight to obvious enhancement. Conclusion The diagnosis of DES should be considered when recurrent masses occur in the extremities,accompanied by obvious hemorrhage,growing along the long bone,multiple cysts or intra-cystic hemorrhage and bone invasion but without obvious structural destruction. Definite diagnosis ultimately relies on pathology.