Malignant peripheral nerve sheath tumors

Gupta, Gaurav; Maniker, Allen H.

doi:10.3171/foc.2007.22.6.13

Cited by 111 publications

(95 citation statements)

References 59 publications

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“…Malignant peripheral nerve sheath tumours are rare and aggressive soft-tissue sarcomas of ectomesenchymal origin [1]. These tumours are derived from Schwann or pleuripotent cells of neural crest origin and arise from peripheral nerve branches or sheaths of peripheral nerve fibres [1][2][3].…”

Section: Discussionmentioning

confidence: 99%

“…These tumours are derived from Schwann or pleuripotent cells of neural crest origin and arise from peripheral nerve branches or sheaths of peripheral nerve fibres [1][2][3]. Malignant peripheral nerve sheath tumours have been referred to by a number of names including ''neurogenic sarcoma'', ''malignant schwannoma'', ''malignant neurilemmoma'' and ''neurofibrosarcoma'' [1,3].…”

Section: Discussionmentioning

confidence: 99%

“…The majority of malignant peripheral nerve sheath tumours occur in patients with neurofibromatosis Type 1 with a cumulative lifetime risk approaching 10% [1]. Malignant peripheral nerve sheath tumours have also been associated with previous radiation therapy [1][2].…”

Section: Discussionmentioning

confidence: 99%

“…Patients with malignant peripheral nerve sheath tumours generally have a poor prognosis because metastatic spread at initial presentation is common [1]. Adverse prognostic factors include large size (.5 cm), high-grade tumour, advanced histological characteristics, tumour invasion of resection margins and neurofibromatosis Type 1 [1].…”

Section: Discussionmentioning

confidence: 99%

“…Adverse prognostic factors include large size (.5 cm), high-grade tumour, advanced histological characteristics, tumour invasion of resection margins and neurofibromatosis Type 1 [1].…”

Section: Discussionmentioning

confidence: 99%

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Malignant peripheral nerve sheath tumour presenting as a pneumothorax

Abbas¹,

Jones²,

Kingston³

et al. 2011

BJR

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ABSTRACT. Malignant peripheral nerve sheath tumours are rare and aggressive softtissue 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 videoassisted 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. Case resportA 34-year-old male presented to our emergency department with acute dyspnoea associated with rightsided pleuritic chest pain. The symptoms had developed suddenly and occurred at rest. There was no history of recent trauma or illness. The patient was a smoker with a 15-pack year history, but had previously been fit and well with no significant medical or family history.On examination, the patient appeared in mild respiratory distress with a respiratory rate of 26 breaths per minute and an oxygen saturation of 91% on room air. He was haemodynamically stable and afebrile. Examination of the respiratory system revealed reduced chest expansion, a hyper-resonant percussion note and reduced air entry over the apex of the right hemithorax. There was no evidence of mediastinal shift. Examination was otherwise unremarkable. Full blood count, biochemistry and electrocardiogram were all normal. A chest radiograph showed a large right-sided pneumothorax associated with a right apical lesion (Figure 1).

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Adverse prognostic factors include large size (.5 cm), high-grade tumour, advanced histological characteristics, tumour invasion of resection margins and neurofibromatosis Type 1 [1].…”

Section: Discussionmentioning

confidence: 99%

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