CAJ | 학술논문

目的分析外周原始神经外胚层肿瘤(pPNET)的MRI表现,并与病理结果相对照.方法收集经病理及免疫组化检查确诊的7例pPNET患者资料,回顾性分析其临床表现、病理特征及MRI表现.结果 7例pPNET中,2例位于颅内,2例位于下肢,1例位于椎管内并跨椎管内外生长,1例位于盆腔内并累及骶骨,1例位于腹膜后.软组织内pPNET呈等T1、稍高T2信号,内可见囊变坏死及出血,1例边缘可见假包膜,增强后肿瘤实性部分明显强化,侵犯周围骨组织.骨pPNET表现为溶骨性骨质破坏,伴有较大的软组织肿块.免疫组化结果显示7例均见CD99表达,4例突触素表达,3例嗜铬粒蛋白表达,2例神经元特异性烯醇酶表达,2例波形蛋白表达,1例上皮细胞膜抗原表达.结论 MRI对外周原始神经外胚层肿瘤的诊断有重要参考价值,确诊仍依赖于病理和免疫组织化学检查.
목적 분석외주원시신경외배층종류(pPNET)적MRI표현,병여병리결과상대조.방법 수집경병리급면역조화검사학진적7례pPNET환자자료,회고성분석기림상표현、병리특정급MRI표현.결과 7례pPNET중,2례위우로내,2례위우하지,1례위우추관내병과추관내외생장,1례위우분강내병루급저골,1례위우복막후.연조직내pPNET정등T1、초고T2신호,내가견낭변배사급출혈,1례변연가견가포막,증강후종류실성부분명현강화,침범주위골조직.골pPNET표현위용골성골질파배,반유교대적연조직종괴.면역조화결과현시7례균견CD99표체,4례돌촉소표체,3례기락립단백표체,2례신경원특이성희순매표체,2례파형단백표체,1례상피세포막항원표체.결론 MRI대외주원시신경외배층종류적진단유중요삼고개치,학진잉의뢰우병리화면역조직화학검사.
Objective To analyze MRI features of peripheral primitive neuroectodermal tumor (pPNET) in comparison with pathological findings. Methods The clinical manifestation, pathological features and the MRI appearances of 7 patients with pathologically proved pPNET were analyzed retrospectively. Results Among 7 patients, 2 patients located in intracalvarium, 2 in lower extremities, 1 in vertebral canal and extended outside of vertebral canal, 1 in cavitas pelvis and involved sacrum, and the rest 1 in the retroperitoneal space. The lesions in soft tissue manifested as iso-intensity on T1WI, slight hyper-intensity on T2WI with cystic degeneration and hemorrhage. Pseudo-capsule was seen in 1 patient. The solid parts of pPNET enhanced markedly after the contrast agent was injected. The adjacent bones were usually involved. pPNET in bone presented as osteolytic destruction of bone with large soft tissue mass. Immunohistochemical examination showed CD99 expression in all 7 patients, synaptophysin (Syn) expression in 4, Chromogranin (CgA) expression in 3, neuron specific enolase (NSE) expression in 2, vimentin (Vim) expression in 2 and EMA in 1 patient. Conclusion MRI has important reference value in diagnosis of pPNET, but final diagnosis still depends on the pathological and immunohistochemical examination.