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European J Pediatr Surg Rep. 2013; 01(01): 005-008
DOI: 10.1055/s-0033-1345105
Case Report
Georg Thieme Verlag KG Stuttgart · New York

Diagnosis and Management of an Isolated Pediatric Plexiform Neurofibroma Involving the Hepatic and Celiac Plexus Using Multimodality Approach: Problem Solving with Diffusion-Weighted Magnetic Resonance Imaging

Merel M. Scheurkogel
1   Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
,
June Koshy
1   Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
,
Kenneth J. Cohen
2   The Johns Hopkins Oncology Center and Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
,
Thierry A. G. M. Huisman
1   Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
,
Thangamadhan Bosemani
1   Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States
› Author Affiliations
Further Information

Publication History

13 January 2013

15 March 2013

Publication Date:
24 April 2013 (online)

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Abstract

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.

Keywords

plexiform neurofibroma - liver - MRI - CT - DWI
 

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