High-frequency ultrasound imaging for cutaneous neurofibroma in patients with neurofibromatosis type I

• Published in: EJD. European journal of dermatology Vol. 27; no. 3; pp. 260 - 265
• Main Authors: Raffin, Delphine, Zaragoza, Julia, Georgescou, Gabriella, Mourtada, Youssef, Maruani, Annabel, Ossant, Frédéric, Patat, Frédéric, Vaillant, Loïc, Machet, Laurent
• Format: Journal Article
• Language: English
• Published: Paris John Libbey Eurotext 01.05.2017
• Subjects: Adolescent; Adult; Aged; Clinical Report; Dermatology; Female; Humans; Life Sciences; Male; Medicine; Medicine & Public Health; Middle Aged; Neurofibromatosis 1 - diagnostic imaging; Neurofibromatosis 1 - pathology; Prospective Studies; Skin Neoplasms - diagnostic imaging; Skin Neoplasms - pathology; Ultrasonography - methods; Young Adult; neurofibromatosis type 1; skin; neurofibroma; ultrasonography; tumour; Prospective Studies; Humans; Middle Aged; Male; Neurofibromatosis 1; Young Adult; Adolescent; Skin Neoplasms; Adult; Female; Aged
• ISSN: 1167-1122; 1952-4013
• DOI: 10.1684/ejd.2017.3015

Background Neurofibromas (NFs) are benign tumours arising from a nerve sheath, which are present in nearly all patients with neurofibromatosis type 1 (NF1). High-frequency ultrasound (HFU) systems, using frequencies over 20 MHz, were developed to improve visualization of skin tumours by means of increased resolution. Objectives To describe NFs by using HFU in patients with NF1. Materials & Methods Anonymized HFU (25-MHz) images of NFs were randomized. Initially, two dermatologist investigators, with experience in HFU imaging of the skin, together described the ultrasound images and established eight criteria for NFs. The same task was then repeated by two other dermatologists, also with experience in HFU imaging of the skin, independently, to establish inter-observer agreement. Results A total of 108 NFs in 29 patients were included. Superficial and subcutaneous NFs were hypoechoic with a round to spindle shape. Plexiform NFs were ill-defined, consisting of multiple hypoechoic linear zones. Good to excellent inter-observer agreement was found for six of the eight criteria (k>0.6). Conclusion This is the first series describing HFU skin imaging of NFs in patients with NF1. Lateral extension that may correspond to involvement of an adjacent nerve seems to be specific to NFs.