Surgical treatment of giant plexiform neurofibroma associated with pectus excavatum

Xuejun Wang,Wenying Liu,Yi Ji,Bing Xu,Siyuan Chen

doi:10.1186/1749-8090-6-119

Abstract

Plexiform neurofibromas are benign tumors originating from subcutaneous or visceral peripheral nerves, which are usually associated with neurofibromatosis type 1. They are almost always congenital lesions and often cause the surrounding soft tissue and bone to grow aberrantly. We treated a 12-year-old boy who presented with asymmetric pectus excavaum and an anterior chest wall plexiform neurofibroma. The pectus excavaum was corrected by modified Nuss procedure, followed by simultaneous resection of the giant mass. The patient is doing well at the 4 years follow-up visit.

Highlights

Plexiform neurofibromas (PNFs) are benign nerve tumor resulting from aberrant growth of the cells of nerve sheath
PNFs have a potential for transformation into highly malignant peripheral nerve sheath tumors, which occur in approximately 5% of patients [2]
PNFs are considered pathognomonic for neurofibromatosis [6]

Summary

Introduction

Plexiform neurofibromas (PNFs) are benign nerve tumor resulting from aberrant growth of the cells of nerve sheath. PNFs have a potential for transformation into highly malignant peripheral nerve sheath tumors, which occur in approximately 5% of patients [2]. There is no accepted effective medical treatment for PNFs. Current management of this disease is limited to surgical resection, but they are usually difficult to completely remove and tend to regrow.

Results

Conclusion