Scheurkogel Merel M, Koshy June, Cohen Kenneth J, Huisman Thierry A G M, Bosemani Thangamadhan
Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States.
The Johns Hopkins Oncology Center and Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland, United States.
European J Pediatr Surg Rep. 2013 Jun;1(1):5-8. doi: 10.1055/s-0033-1345105. Epub 2013 Apr 24.
Plexiform neurofibroma with involvement of the gastrointestinal tract is a very rare entity in children. Here, we present a rather unique case of a 9-year-old boy with no clinical signs or features of neurofibromatosis type 1. A periportal mass lesion was incidentally found after performing an ultrasound in this previously healthy child. Computed tomographic scan was subsequently performed which showed a low-density mass in a periportal distribution with extension along the celiac axis. Because the findings were nonspecific, a pre- and postcontrast magnetic resonance imaging of the abdomen was performed which included diffusion-weighted imaging. The lesion was then confirmed to be a plexiform neurofibroma with open biopsy. Management of plexiform neurofibromas varies widely. Given the extensive nature of the lesion, managing the patient with follow-up rather than surgical excision was favored.
累及胃肠道的丛状神经纤维瘤在儿童中是一种非常罕见的疾病。在此,我们报告一例颇为独特的病例,一名9岁男孩,无1型神经纤维瘤病的临床体征或特征。在对这名此前健康的儿童进行超声检查时偶然发现了一个肝门周围的肿块病变。随后进行了计算机断层扫描,显示肝门周围分布有低密度肿块,并沿腹腔干延伸。由于检查结果不具有特异性,于是对腹部进行了增强前后的磁共振成像检查,其中包括扩散加权成像。随后通过开放性活检证实该病变为丛状神经纤维瘤。丛状神经纤维瘤的治疗方法差异很大。鉴于病变范围广泛,更倾向于对患者进行随访观察而非手术切除。