A LARGE MALIGNANT PERIPHERAL NERVE SHEATH (MPNST) OF RADIAL NERVE- A RARE CASE REPORT.

Malignant peripheral nerve sheath tumour (MPNST) are rare, aggressive sarcomatous tumours of peripheral nerve sheaths which can arise from preexisting neurofibroma or de novo.Sporadic origin accounts for nearly half of all MPNST cases,while other cases occur in association with neurofibromatosis type 1 (NF1).The most common site is nerve trunks of extremities like sciatic nerve. On literature search only four case reports of MPNST of radial nerve are published till date.We report a case of 50 years old lady presenting with a large,firm,ovoid swelling measuring 7 cm x 8 cm over her right arm with symptoms of neuropathy.The fine needle aspiration cytology report was a benign neurogenenic tumour. MRI reported as a radial nerve sheath tumour. Core biopsy shows features of neurofibroma(NF).She was subjected to treatment as a benign pathology but the final histopathological report was a low grade - malignant peripheral nerve sheath tumour.

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Management of an incidental malignant peripheral nerve sheath tumour in the parapharyngeal space

The Journal of Laryngology & Otology ◽

10.1017/s0022215112002459 ◽

2012 ◽

Vol 127 (1) ◽

pp. 104-106 ◽

Cited By ~ 2

Author(s):

E Mushi ◽

S Winter

Keyword(s):

Peripheral Nerve ◽

Neurofibromatosis Type 1 ◽

Parapharyngeal Space ◽

Incidental Finding ◽

Neurofibromatosis Type ◽

Low Grade ◽

Nerve Sheath Tumour ◽

Nerve Sheath ◽

Peripheral Nerve Sheath Tumour

AbstractBackground:Parapharyngeal space tumours are uncommon and represent 0.5–1 per cent of all head and neck neoplasms; 20–30 per cent of these are malignant. Malignant peripheral nerve sheath tumours are rare and mostly encountered in patients with neurofibromatosis type 1. Only four cases of parapharyngeal space tumours have been reported in the English language in patients without neurofibromatosis type 1.Case report:We report the case of a 64-year-old man with no stigmata of neurofibromatosis type 1, in whom a mass in the left pre-styloid region of the parapharyngeal space was an incidental finding following magnetic resonance imaging for investigation of cervical spine problems. The mass was consequently removed using a transcervical approach. A histological review revealed a low-grade malignant peripheral nerve sheath tumour.Discussion:We consider the pathophysiology of this highly malignant tumour as well as the challenging anatomy of the parapharyngeal space and the surgical and other therapeutic modalities utilised to treat this condition.

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An unusual presentation of intraosseous malignant peripheral nerve sheath tumour of mandible

Dentomaxillofacial Radiology ◽

10.1259/dmfr.20180341 ◽

2019 ◽

Vol 48 (7) ◽

pp. 20180341 ◽

Cited By ~ 1

Author(s):

Sanghoon Lee ◽

Chena Lee ◽

Jin-kyu Kim ◽

Woong Nam

Keyword(s):

Peripheral Nerve ◽

Signal Intensity ◽

Inferior Alveolar Nerve ◽

Magnetic Resonance Images ◽

Low Grade ◽

Nerve Sheath Tumour ◽

Nerve Sheath ◽

Grade Malignancy ◽

Inferior Alveolar Nerve Canal ◽

Peripheral Nerve Sheath Tumour

Malignant peripheral nerve sheath tumour (MPNST) is a rare mesenchymal tumour which usually presents high grade malignancy. We report an atypical case of intraosseous malignant peripheral nerve sheath tumour on mandible in a 36-year-old male. Patient presented with an incidentally discovered radiolucency on the left anterior mandible and did not complain of any symptoms. Panoramic radiograph and computed tomography showed enlargement of inferior alveolar nerve canal, thinning of the cortex and calcified foci within an expansile mass. Magnetic resonance images revealed heterogeneous hyperintense signal intensity with a well-defined margin on T2 weighted images, heterogeneous enhancement on contrast-enhanced T1 weighted images and intermediate signal intensity and inferior alveolar nerve canal enlargement on T1 weighted images. In spite of benign radiologic characteristics as mentioned above, histopathologic examination of biopsied specimen otherwise revealed a low-grade malignancy. Wide excision of mandible was performed and there has been no loco-regional recurrence or distant metastasis over 1 year following surgery. This case indicates that, even when imaging modalities clearly demonstrate benign nature of intraosseous neurogenic tumour, care must be taken to establish proper treatment plan for assumed malignancy with a definitive histopathological analysis.

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