Peripheral nerve tumours

This chapter on peripheral nerve sheath tumours (PNSTs) explores the pathology, clinical presentation, investigation, management, and outcomes of both benign and malignant peripheral nerve sheath tumours (MPNSTs). The focus is centred upon the more commonly encountered tumours: schwannoma, neurofibroma, and perineurioma. Significant emphasis is also given to the rarer but life-threatening MPNSTs. It aims to equip the reader with the latest information available in the literature with regards to PNSTs occurring sporadically or in association with inherited conditions. Up to date investigation and management options available are also presented. With the detailed insights provided, the aim is to ensure that the reader would be confident in managing patients presenting with peripheral nerve tumours.

Tumours of cranial and peripheral nerves

Chapter 8.17 covers tumours of cranial and peripheral nerves, including vestibular schwannoma (VS), neurofibroma, and malignant peripheral nerve tumours (MPNSTs).

VON RECKLINGHAUSEN'S DISEASE

Von Recklinghausen's disease (VRD) is a rare genetic autosomal dominant disorder that affects the ectodermaltissues (nerve and skin). The cardinal features of this disorder are spots of increase pigmentation (cafe-au-lait spot, CAL), peripheral nerve tumours (neurofibromatosis) and iris hamartoma (Lisch nodule). Clinicaldiagnosis of VRD is based on the criteria given by National Institute of Health (NIH) because the mutationanalysis is laborious. Here, we are reporting a case of a family with VRD.Key Words: VonRecklinghausen's disease (VRD), Cafe-au-lait (CAL) spot, Lisch nodule,Neurofibromatosis (NF)