Appearance and clinical value of MRI in sinomasal embryonal rhabdomyosarcoma

　　鼻腔鼻窦胚胎型横纹肌肉瘤磁共振表现及临床价值

　　Abstract：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 multi location. 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.

　　目的 探讨诊断鼻腔和鼻旁窦胚胎型横纹肌肉瘤表现和核磁共振的临床价值。方法回顾分析5位鼻腔和鼻旁窦胚胎型横纹肌肉瘤患者的临床，病理和核磁共振的发现。结果多数临床表现包括鼻塞（4例），眼球突出（4例），视力下降(2例)，鼻出血（1例），嗅觉下降（1例）及眼球运动障碍（1例）。5例中4例发生在筛窦，1例主要发生在鼻中隔。依据国际横纹肌肉瘤研究组(Intergroup Rhabdomyosarcoma Studies,IRS)分期标准1例处于Ⅱ期，其他Ⅲ期。和脑灰质比较病变，2例T1W1为均匀等信号。3例T1W1高信号等强度。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.