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1型神经纤维瘤病中的髓质不明亮物体:病例系列

Medullary unidentified bright objects in Neurofibromatosis type 1: a case series.

作者信息

D'Amico Alessandra, Mazio Federica, Ugga Lorenzo, Cuocolo Renato, Cirillo Mario, Santoro Claudia, Perrotta Silverio, Melis Daniela, Brunetti Arturo

机构信息

Department of Advanced Biomedical Sciences, "Federico II" University, via Sergio Pansini 5, 80100, Naples, Italy.

, Naples, Italy.

出版信息

BMC Pediatr. 2018 Feb 28;18(1):91. doi: 10.1186/s12887-018-1067-1.

Abstract

BACKGROUND

In Neurofibromatosis type 1, cerebral Unidentified Bright Objects are a well-known benign entity that has been extensively reported in the literature. In our case series, we wish to focus on a further possible location of such lesions, the spinal cord, which we have defined as medullary Unidentified Bright Objects. These have been, to our knowledge, scarcely described in previous works.

CASE PRESENTATION

We report the cases of 7 patients with medullary Unidentified Bright Objects in Neurofibromatosis type 1 that we have followed for up to 9 years in our Regional Referral Center for Neurofibromatosis. In all of our patients, these lesions were completely asymptomatic and reported on Magnetic Resonance exams the patients underwent for other clinical indications.

CONCLUSIONS

The aim of our work is to increase awareness of the possibility of medullary Unidentified Bright Objects in Neurofibromatosis type 1 patients, which can simulate neoplastic lesions, suggesting a more conservative approach in these cases.

摘要

背景

在1型神经纤维瘤病中,脑内不明高信号灶是一种广为人知的良性病变,文献中已有大量报道。在我们的病例系列中,我们希望关注此类病变的另一个可能位置——脊髓,我们将其定义为髓内不明高信号灶。据我们所知,此前的研究很少描述这些病变。

病例报告

我们报告了7例1型神经纤维瘤病患者髓内不明高信号灶的病例,这些病例在我们地区神经纤维瘤病转诊中心接受了长达9年的随访。在我们所有的患者中,这些病变完全无症状,是在患者因其他临床指征接受磁共振检查时发现的。

结论

我们这项工作的目的是提高对1型神经纤维瘤病患者出现髓内不明高信号灶可能性的认识,这些病灶可能类似肿瘤性病变,提示在这些病例中应采取更保守的方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9d82/5832195/7c23600dc97e/12887_2018_1067_Fig1_HTML.jpg

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