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神经纤维瘤病 1 型中所见的腹盆腔神经肿瘤的放射学表现。

Radiological manifestations of abdominopelvic nerve tumours seen in neurofibromatosis type 1.

机构信息

Department of Radiology, Division of Pediatric Radiology, Hacettepe University Medical School, Ankara, Turkey.

Department of Radiology, Hacettepe University Medical School, Ankara, Turkey.

出版信息

Clin Radiol. 2014 Apr;69(4):431-5. doi: 10.1016/j.crad.2013.11.007. Epub 2013 Dec 19.

Abstract

Neurofibromatosis type 1 (NF1) is the most common of the phakomatoses, which is inherited in an autosomal dominant pattern. Neurofibromas arise from Schwann cells and fibroblasts and plexiform neurofibromas are pathognomonic for NF1, which may arise in any peripheral nerve. The clinical expression of NF1 is extremely variable and gastrointestinal manifestations of NF1 are relatively uncommon. The purpose of this article is to illustrate the abdominal ultrasound, computed tomography, and magnetic resonance imaging findings of abdominopelvic nerve tumours seen in NF1 in both paediatric and adult patients.

摘要

神经纤维瘤病 1 型(NF1)是神经皮肤疾病中最常见的一种,呈常染色体显性遗传。神经纤维瘤起源于施万细胞和成纤维细胞,丛状神经纤维瘤是 NF1 的特征性表现,可发生于任何外周神经。NF1 的临床表现极其多样,胃肠道表现相对少见。本文旨在阐述 NF1 患者的腹部超声、计算机断层扫描和磁共振成像结果,以展示其腹盆腔神经肿瘤的影像学特征,包括儿童和成人患者。

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