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

2017 ◽  
Vol 4 (10) ◽  
pp. 3552
Author(s):  
Shahaji Chavan ◽  
Shubhi P. Bhatnagar ◽  
Mahendra Bendre ◽  
Anuradha Dnyanmote ◽  
Vinayak Kshirsagar ◽  
...  

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. 

2019 ◽  
Vol 48 (7) ◽  
pp. 20180341 ◽  
Author(s):  
Sanghoon Lee ◽  
Chena Lee ◽  
Jin-kyu Kim ◽  
Woong Nam

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.


2015 ◽  
Vol 04 (05) ◽  
pp. 872-878
Author(s):  
Sampath Kumar K ◽  
Rajesh Kumar A ◽  
Neeraja M ◽  
Venkata Ramananababu P

2014 ◽  
Vol 22 (3) ◽  
pp. 409-414 ◽  
Author(s):  
Piya Kiatisevi ◽  
Chaiwat Piyaskulkaew ◽  
Bhasanan Sukunthanak ◽  
Voranuch Thanakit ◽  
Saraporn Bumrungchart

2008 ◽  
Vol 2 (6) ◽  
pp. 637 ◽  
Author(s):  
Julie O’Brien ◽  
Susan Aherne ◽  
Orla Buckley ◽  
Padraig Daly ◽  
William C. Torreggiani

Neurofibromatosis is a hamartomatous disorder of autonomic peripheral nerve sheaths associated with peripheral nerve sheath tumours. Most tumours are neurofibromas; however, the genitourinary system is rarely involved. We present a rare case of a nerve sheath tumour of the bladder in a young patient, which was discovered to be malignant.


2012 ◽  
Vol 127 (1) ◽  
pp. 104-106 ◽  
Author(s):  
E Mushi ◽  
S Winter

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.


Open Medicine ◽  
2012 ◽  
Vol 7 (5) ◽  
pp. 655-658
Author(s):  
Elvyra Voluckiene ◽  
Giedrius Uzdavinys ◽  
Saulius Cicenas ◽  
Loreta Ivaskeviciene ◽  
Diana Zakarkaite ◽  
...  

AbstractA malignant peripheral nerve sheath tumour (MPNST) is a rare variety of soft-tissue sarcoma of ectomesenchymal origin. The World Health Organisation created the term MPNST to replace previous terminology such as malignant schwannoma, malignant neurilemmoma, neurogenic sarcoma, and myxofibrosarcoma for tumours of neurogenic origin with similar biological behavior.[1–3] The vast majority of these tumours develop in extremities. They also tend to be located in unusual sites of the body, such as the pelvic retroperitoneum, infratemporal fossa, intrapericardium, and mediastinum.[1,3,4] This case study presents a patient with an extremely rare primary cardiac MPNST..


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