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An Unusual Presentation of Intraosseous Malignant Peripheral Nerve Sheath Tumour of Mandible

 Sanghoon Lee  ;  Chena Lee  ;  Jin-Kyu Kim  ;  Woong Nam 
 DENTOMAXILLOFACIAL RADIOLOGY, Vol.48(7) : e20180341, 2019-10 
Journal Title
Issue Date
Adult ; Humans ; Magnetic Resonance Imaging ; Male ; Mandible* / diagnostic imaging ; Mandible* / surgery ; Nerve Sheath Neoplasms* / diagnostic imaging ; Nerve Sheath Neoplasms* / pathology ; Nerve Sheath Neoplasms* / surgery ; Neurofibrosarcoma* / diagnostic imaging ; Neurofibrosarcoma* / pathology ; Neurofibrosarcoma* / surgery ; Treatment Outcome
head and neck tumor ; malignant peripheral nerve sheath tumor ; malignant schwannoma ; neurofibrosarcoma ; neurogenic sarcoma
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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2. College of Dentistry (치과대학) > Dept. of Oral and Maxillofacial Surgery (구강악안면외과학교실) > 1. Journal Papers
2. College of Dentistry (치과대학) > Dept. of Oral and Maxillofacial Radiology (영상치의학교실) > 1. Journal Papers
Yonsei Authors
Kim, Jin-Kyu(김진규)
Nam, Woong(남웅) ORCID logo https://orcid.org/0000-0003-0146-3624
Lee, Chena(이채나) ORCID logo https://orcid.org/0000-0002-8943-4192
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