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European J Pediatr Surg Rep. 2013 Jun;1(1):5-8. doi: 10.1055/s-0033-1345105. Epub 2013 Apr 24.

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.

European journal of pediatric surgery reports

Merel M Scheurkogel, June Koshy, Kenneth J Cohen, Thierry A G M Huisman, Thangamadhan Bosemani

Affiliations

  1. Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland, United States.
  2. The Johns Hopkins Oncology Center and Department of Pediatrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland, United States.

PMID: 25755938 PMCID: PMC4336049 DOI: 10.1055/s-0033-1345105

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: CT; DWI; MRI; liver; plexiform neurofibroma

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