Fig. 10
From: Masses of developmental and genetic origin affecting the paediatric craniofacial skeleton
Rapidly enlarging painful plexiform neurofibroma (PNF) in a 15-year-old girl with neurofibromatosis type 1 (NF1). Imaging was performed before PNF resection. a Panoramic view shows mandible deformity (thinning and bowing of the right ascending ramus of the mandible and the right mandibular body) with involvement and enlargement of the right inferior alveolar nerve canal (arrow). b The axial T2 images reveal extensive PNF (asterisks) with a characteristic “target sign”: central T2 hypointensity within the multilobulated hyperintense mass (arrowheads). c Gadolinium-enhanced coronal fat-saturated T1 shows large, infiltrative and multilobulated PNF involving the entire hemiface with perineural spread and extension to the right foramen ovale (arrow). d Immunohistochemistry (original magnification, ×40; S100 protein) obtained from surgical specimen highlights plexiform nerve bundles (arrows) dissecting the adipose tissue of the hypodermis (asterisk). Numerous confluent Wagner-Meissner bodies are seen (inset, original magnification, ×200; H&E stain)