Neurofibrosarcoma of the mandible derived from neurofibromatosis

Autopsy and Case Reports

卷:9

Viviana Lanel¹  Marcelo Marcucci¹  Juliane Pirágine Araujo²  Jefferson Xavier de Oliveira² 

[1] Hospital Heliopolis, Department of Stomatology;

[2] Universidade de São Paulo, School of Dentistry, Department of Stomatology;

关键词: Neurofibromatosis 1;    Neurofibrosarcoma;    Neurilemmoma;   

DOI  :  10.4322/acr.2019.094

来源: DOAJ

【 摘 要 】

Neurofibrosarcoma is a rare malignant neoplasm of the head and neck region and accounts for 8% to 16% of all cases. Its origin is varied and may stem from cells of the peripheral nerves, develop de novo, or result from malignant transformation of preexisting neurofibromas. Because the features of neurofibrosarcomas are heterogeneous, the data retrieved during clinical examinations are of great aid for diagnosis. In this case, owing to clinical features and the fact that the patient had neurofibromatosis type 1, the hypothesis of neurofibrosarcoma was promptly established. The final diagnosis was confirmed by associating clinical, imaging, and pathological data. After the treatment, the patient has been followed up for 10 years, with no evidence of recurrence.

【 授权许可】

Unknown