Neurofibrosarcoma of the mandible derived from neurofibromatosis

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 ne...

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Bibliographic Details
Published in:Autopsy & case reports 2019, Vol.9 (4)
Main Authors: Araujo, Juliane Pirágine, Oliveira, Jefferson Xavier de, Lanel, Viviana, Marcucci, Marcelo
Format: Article
Language:English
Subjects:
Clinical Case Report
Neurilemmoma
Neurofibromatosis 1
Neurofibrosarcoma
Online Access:Get full text
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Summary: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.
ISSN:2236-1960
2236-1960
DOI:10.4322/acr.2019.094