鼻腔鼻窦胚胎型横纹肌肉瘤磁共振表现及临床价值

摘要

目的 探讨鼻腔鼻窦胚胎型横纹肌肉瘤(embryonal rhabdomyosarcoma,ERMS)的磁共振(MRI)表现和临床价值.方法 回顾性分析5例经组织病理学证实的鼻腔鼻窦ERMS的临床、病理和MRI资料,重点分析肿瘤发生的年龄、临床症状、发生部位和MRI特点,以及MRI在诊断和治疗过程中的价值.结果 ERMS患者主要临床症状包括:鼻塞4例次,眼球突出4例次,视力下降2例次,涕中带血1例次,嗅觉下降1例次,眼球活动受限1例次.本组5例中,有4例病变主体位于筛窦,1例位于鼻中隔,均累及眼眶、前颅窝底脑膜及多个鼻窦.依据国际横纹肌肉瘤研究组(Intergroup Rhabdomyosarcoma Studies,IRS)分期标准,Ⅱ期1例,Ⅲ期4例.病变与脑灰质比较,T1W1为均匀等信号2例;以等信号为主,散在小片状高信号3例.T2W1以稍高信号为主,混杂片状低信号2例;以稍高信号为主,见散在小点状、片状更高信号3例.5例ERMS增强扫描均为明显不均匀强化,内见散在片状无强化区,其中2例见线环样、葡萄样强化.4例肿瘤内有出血.全部5例均有不同程度的骨质推压改变和骨质破坏,骨质破坏区均见残存骨质.结论 鼻窦ERMS的MRI表现具有一定的特征性,MRI能准确显示肿瘤的侵犯范围,对肿瘤的定性诊断、临床分期和治疗效果的评价均有一定的指导意义.%Objective To investigate the appearance and clinical value of MRI in the diagnosis of embryonal rhabdomyosarcoma in nasal cavity and paranasal sinuses. Methods The clinical, pathological and MRI findings of five patients with pathology proved embryonal rhabdomyosarcoma in nasal cavity and paranasal sinuses were retrospectively analyzed. Results The major clinical symptoms included nasal obstruction (4 cases) , exophthalmus (4 cases) , decreased eyesight (2 cases), epistaxis ( 1 case) , decreased olfactory sensation (1 case) and restriction of eyeball movement (1 case). All 5 cases involved multilocation. Among them, 4 cases mainly located in the ethmoid sinus, one mainly located in the nasal septum. According to the IRS, 1 case was at stage Ⅱ , the other 4 cases were at stage Ⅲ. Compared to the grey matters, on T1 weighted image, the masses were homogenously isointensity in 2 cases, isointensity with patchy hyperintensity in 3 cases. On T2 weighted images, the masses were slightly hyperintensity with patchy hypointensity in 2 cases. Slightly hyperintensity with patchy hyperintensity in 3 cases. All 5 cases were markly heterogeneously enhanced after administration of contrast agents, with patchy of non-enhanced area. Of them, two were grape-like enhanced. Four cases presented with intratumor hemorrhage. Five with bony destruction. All of the 5 cases showed orbits and anterior cranial fossa meningeal involvement. Conclusions There are some special MRI findings in embryonal rhabdomyosarcoma located in nasal cavity and paranasal sinuses. MRI can depict the encroachment of the tumor accurately, and may play an important role in clinical staging and in curative effect evaluation.