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

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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A CASE REPORT OF LOW GRADE MALIGNANT PERIPHERAL NERVE SHEATH TUMOUR

Journal of Evolution of Medical and Dental Sciences ◽

10.14260/jemds/2015/124 ◽

2015 ◽

Vol 04 (05) ◽

pp. 872-878

Author(s):

Sampath Kumar K ◽

Rajesh Kumar A ◽

Neeraja M ◽

Venkata Ramananababu P

Keyword(s):

Case Report ◽

Peripheral Nerve ◽

Low Grade ◽

Nerve Sheath Tumour ◽

Nerve Sheath ◽

Peripheral Nerve Sheath Tumour

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Low grade malignant peripheral nerve sheath tumour: varied cytological and histological patterns

Journal of Clinical Pathology ◽

10.1136/jcp.56.11.826 ◽

2003 ◽

Vol 56 (11) ◽

pp. 826-830 ◽

Cited By ~ 24

Author(s):

U Yamaguchi

Keyword(s):

Peripheral Nerve ◽

Low Grade ◽

Nerve Sheath Tumour ◽

Nerve Sheath ◽

Peripheral Nerve Sheath Tumour

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Total Sacrectomy for Low-Grade Malignant Peripheral Nerve Sheath Tumour: A Case Report

Journal of Orthopaedic Surgery ◽

10.1177/230949901402200328 ◽

2014 ◽

Vol 22 (3) ◽

pp. 409-414 ◽

Cited By ~ 2

Author(s):

Piya Kiatisevi ◽

Chaiwat Piyaskulkaew ◽

Bhasanan Sukunthanak ◽

Voranuch Thanakit ◽

Saraporn Bumrungchart

Keyword(s):

Case Report ◽

Peripheral Nerve ◽

Low Grade ◽

Nerve Sheath Tumour ◽

Nerve Sheath ◽

Total Sacrectomy ◽

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

10.36106/paripex/8400904 ◽

2020 ◽

pp. 1-3

Author(s):

Dhanabir Thangjam ◽

L. Chittaranjan Singh ◽

Ramthaipou Kamei ◽

Nehar Sinam ◽

M. Amitkumar

Keyword(s):

Peripheral Nerve ◽

Radial Nerve ◽

Case Reports ◽

Neurofibromatosis Type ◽

Needle Aspiration ◽

Low Grade ◽

Nerve Sheath Tumour ◽

Nerve Sheath ◽

Rare Case Report ◽

Peripheral Nerve Sheath Tumour

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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Malignant peripheral nerve sheath tumour over the posterior triangle of neck: a rare case report

International Surgery Journal ◽

10.18203/2349-2902.isj20174538 ◽

2017 ◽

Vol 4 (10) ◽

pp. 3552

Author(s):

Shahaji Chavan ◽

Shubhi P. Bhatnagar ◽

Mahendra Bendre ◽

Anuradha Dnyanmote ◽

Vinayak Kshirsagar ◽

...

Keyword(s):

Soft Tissue ◽

Peripheral Nerve ◽

Rare Case ◽

The Body ◽

Surgical Removal ◽

Low Grade ◽

Nerve Sheath Tumour ◽

Nerve Sheath ◽

Peripheral Nerve Sheath Tumour

Malignant peripheral nerve sheath tumour (MPNST) is an extremely rare soft tissue sarcoma which usually arises from peripheral nerves or somatic soft tissue with an incidence of 0.001%. It’s most common anatomical sites are the proximal portions of the upper and lower extremities and the trunk and it’s extremely rare for such a tumour to occur elsewhere in the body. We report a rare case of such a tumour over the left posterior aspect of neck in a 28-year-old female patient. We have reviewed this case in terms of clinical presentation, investigations, surgical treatment and adjuvant therapy and have shortly described our experience. MRI and CT neck supported the diagnosis of this tumour. Fine needle aspiration cytology taken from the swelling revealed a low-grade spindle cell tumour with a possibility of MPNST. Excision of the tumour was done and the excised specimen was sent for histopathological examination which revealed MPNST. Adjuvant radiotherapy was given postoperatively. At a 6-month follow-up, patient is doing well with no evidence of recurrence. Suspicion of this tumour should be raised in a rapidly growing painless tumour in and around a nerve tissue. Diagnosis is made by assessing a combination of clinical, pathological and immunohistochemistry features. Complete surgical removal should be the goal of treatment with definitive histological diagnosis. A regular follow up is recommended to confirm any recurrence or metastasis.

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