title: Malignant peripheral nerve sheath tumour presenting as a pneumothorax.
abstract: Malignant peripheral nerve sheath tumours are rare and aggressive soft-tissue sarcomas of ectomesenchymal origin. These tumours commonly occur in patients with neurofibromatosis Type 1 with a cumulative lifetime risk of 10%. The vast majority of cases present with clinical evidence of a soft-tissue mass with or without features of nerve irritation and loss of function arising from the lesion of origin. The primary presentation of a malignant peripheral nerve sheath tumour with a pneumothorax in the absence of widespread metastatic disease in a patient with no medical or family history of neurofibromatosis has never been reported in the literature. We present a unique case of a systemically well 34-year-old male who presented with clinical evidence of a right-sided pneumothorax. The chest radiograph identified the right-sided pneumothorax and revealed an apical pleural mass that was confirmed by intravenous contrast-enhanced CT of the thorax. The patient was referred for video-assisted thorascopic surgical pleurodesis and biopsy of the lesion. Histopathology analyses confirmed the diagnosis of malignant peripheral nerve sheath tumour. To the best of our knowledge, no such case reports have been published in the literature. A diagnosis of malignant peripheral nerve sheath tumour should be considered as one of the rarer possibilities in patients presenting with pneumothoraces in association with apical pleural lesions.
authors.affiliation: Radiology department, Southampton University Hospitals NHS Trust, Southampton, Hampshire, UK. drausami_abbas@msn.com
authors.forename: A
authors.initials: A
authors.lastname: Abbas
authors.forename: H
authors.initials: H
authors.lastname: Jones
authors.forename: G T
authors.initials: GT
authors.lastname: Kingston
authors.forename: A
authors.initials: A
authors.lastname: Zurek
mesh_terms: D000328:Adult; D000409:Alanine / Q000031:analogs & derivatives; D000970:Antineoplastic Agents / Q000008:administration & dosage; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D018317:Nerve Sheath Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology; D010524:Peripheral Nervous System Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging* / Q000188:drug therapy / Q000473:pathology; D011030:Pneumothorax / Q000000981:diagnostic imaging / Q000209:etiology* / Q000473:pathology; D011758:Pyrroles / Q000008:administration & dosage; D013902:Radiography, Thoracic; D014227:Triazines / Q000008:administration & dosage
chemical_list: D000970:Antineoplastic Agents; D011758:Pyrroles; D014227:Triazines; C509922:brivanib; D000409:Alanine