神经纤维瘤病Ⅰ型腹部受累影像学表现的研究

Imaging Appearances of Neurofibromatosis Ⅰ with Abdominal Region Involved

目的探讨神经纤维瘤病Ⅰ型(NFI)腹部受累的影像学表现,旨在进一步提高对本病的认识。方法回顾性分析8例经手术或临床和影像学表现证实的神经纤维瘤病Ⅰ型,8例术前均行CT平扫加增强,3例行MRI检查。结果为多结构受累:盆腔受累5例,腹腔受累3例,伴累及后腹壁5例,臀部肌间隙、骶管旁、骶前间隙3例,脊柱旁、腰大肌1例。病灶呈分叶状5例,迂曲血管状2例,"哑铃状"1例,病灶数目较多者呈"葡萄串状"。病变大小不等。CT平扫均呈混杂软组织密度,其中中央为低密度区7例,为高密度区1例;增强扫描可见病灶轻度强化4例,明显强化3例,强化不明显1例。MR平扫时T1WI均呈等低信号,T2WI呈中央高信号1例,中央低信号2例,增强可见轻度不均匀强化。邻近脏器受压、推移,脊柱向对侧侧弯2例,膀胱、直肠壁增厚者2例,小肠壁毛糙不光整者2例,椎孔、骶骨孔扩大者3例。结论神经纤维瘤病Ⅰ型腹部受累的影像表现多样化,主要为间质瘤及丛状神经纤维瘤,而丛状神经纤维瘤主要见于腹壁和腰骶丛,间质瘤多生长于消化道附近。结合临床和其他部位影像学资料,可提高其诊断符合率。

Objective To explore the imaging appearances of neurofibromatosis I （NFI） with the abdominal region involved, thus to improve cognition of this disease further. Methods Retrospective study was done on 8 cases of NFI proved by operation or clinical and imaging appearances, and all these 8 cases underwent CT plain/enhanced scans while 3 cases among these underwent MRI scans before operation. Results There were multiple body structure in volvement： pelvic in 5 cases, abdomen involment in 3 cases,retroperitoneal in- volvement in 5 cases, glutaeus space, para - sacral canal and presacral space involment in 3 cases, para - spinal column and psoas major involment in 1 case. Lesions with lobulated appearances were found in 5 cases, tortuous vascular appearances in 2 cases, dumbbell - loo- king in 1 case. And when the lesions became more, there were grape clusters looking, lesions were inequality of size. CT plain scans showed mingle soft - tissue density, with low density in centre in 7 cases, high density in 1 case. MRI plain scans showed low equal sig- nals, with high signals in centre in 1 case, low signals in centre in 2 cases. MRI enhaneemengt scans showed mild inhomogeneous strengthen. Adjacent organs were pressed and pushed,and spinal cords were edge bended to the other side. Thickening of bladder and rec- tal wall was found in 2 cases, rough and unfitting of small intestine walls in 2 cases, enlargement of vertebral and sacral foramen in 3 ca- ses. Conclusion The imaging appearances showed diversify in NFI abdomen involvement, with interstitialoma and Plexiform neurofibro-ma mainly. Plexiform neurofibroma was seen in abdominal wall and lumbosacral plex more,while interstitialoma frequently vegetated near primae viae. Diagnostic accuracy could be raised when combined use of clinical and other regions imaging data.