Benign and malignant tumors are common in the setting of neurofibromatosis type 1 (NF1). Malignant peripheral nerve sheath tumor (MPNST) and angiosarcoma are rare tumors in children and adolescents and mostly occur in young patients in relation to NF1. Both histological types can be present in the same tumor mass in patients with NF1. We present a case of 12.5-year-old girl with NF1 who first p...

Malignant peripheral nerve sheath tumours (MPNST) are a group of malignant tumours that arise from cells that are differentiating or have differentiated toward those of the peripheral nerve sheath.1 The heterogeneity of these cellular components and stages of differentiation have historically lead to difficulty in defining the diagnosis and behaviour of these lesions, with terms such as maligna...

We present the diŠusion-weighted imaging (DWI) ˆndings for a malignant peripheral nerve sheath tumor arising in a retroperitoneal plexiform neuroˆbroma in a patient with neuroˆbromatosis type 1. Signal intensity of the malignant area was high on DWI and low on the apparent diŠusion coe‹cient map and diŠered from ˆndings for the benign area. DWI enabled clear diŠerentiation between malignant and...

A young male presented with clinical and radiological features of right apical lung mass and Horner's syndrome. Subsequently the patient was diagnosed as a case of malignant peripheral nerve sheath tumour (MPNST) at the apex of right lung originating from an intercostal nerve and compressing ipsilateral cervical sympathetic plexus and lower cord of brachial plexus, in a case of neurofibromatosi...

Schwannomas, also known as neurilemmomas, are generally benign peripheral nerve sheath tumors developing from Schwann cells. Peripheral nerve sheath tumors account for less than 8% of soft tissue neoplasms. Schwannomas are characterized by a slow-growing and non-infiltrating pattern. We report a 21-year-old, right-handed male, with a mass at his right elbow anterolateral region, that was slowly...

Ewing's sarcoma is a common bone tumour of childhood but is a rare occurrence in individuals over 20 years of age. Few cases are reported as originating from peripheral nerves. We present an unusual case of extraosseous Ewing's sarcoma originating from the sciatic nerve in a 66-year-old patient which had the clinical hallmarks of a benign nerve sheath tumour. Following discussion at a multidisc...

The aim of our study was to document whether relationships existed among bone morphogenetic proteins (BMPs), peripheral nerve and neoplastic lesions of nerve sheath tumors. The mRNA transcriptions of BMP-2, 3, 4 and 5 in 10 cases of schwannoma, three cases of malignant schwannoma and two cases of trigeminal neuralgia were detected using an in situ hybridization technique. Our results demonstrat...