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

• Main Authors: Merel M. Scheurkogel (Author), June Koshy (Author), Kenneth J. Cohen (Author), Thierry A. G. M. Huisman (Author), Thangamadhan Bosemani (Author)
• Format: Book
• Published: Georg Thieme Verlag KG, 2013-04-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 2194-7619; 2194-7627; 10.1055/s-0033-1345105
• Keywords: plexiform neurofibroma; liver; mri; ct; dwi; Pediatrics; RJ1-570; Surgery; RD1-811
• Source: European Journal of Pediatric Surgery Reports, Vol 01, Iss 01, Pp 005-008 (2013)

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.