Malignant peripheral nerve sheath tumour (MPNST) of thorax: a rare soft tissue sarcoma in a patient with neurofibromatosis type 1

Neurofibromatosis is a genetic disorder with multisystem involvement. Malignant peripheral nerve sheath tumours (MPNST) are one of the soft tissue sarcomas associated with neurofibromatosis type1 (NF-1). These tumours are relatively rare and mostly occur in proximal portions of the upper and lower extremities and the trunk. MPNST of the thoracic cavity has rarely been reported. This case report describes a woman with NF-1 who presented with progressive dyspnoea and was found to have a mass in the thoracic cavity. She underwent partial resection and was found to have MPNST. Intrathoracic MPNST have a bad prognosis often needing neoadjuvant chemotherapy or chemoradiation pre/ post resection.