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Case Report
Malignant peripheral nerve sheath tumor of the infratemporal fossa: Case report and literature review
1 ENT Resident, Department of Otorhinolaryngology Head and Neck Surgery, Centro Hospitalar Universitário de Lisboa Central, Lisbon, Portugal
2 ENT Senior Consultant, Department of Otorhinolaryngology Head and Neck Surgery, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal
3 Pathological Anatomy Resident, Department of Pathological Anatomy, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal
4 Radiation Oncology Consultant, Department of Radiation Oncology, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal
5 Head of ENT Department, Department of Otorhinolaryngology Head and Neck Surgery, Instituto Português de Oncologia de Lisboa Francisco Gentil, Lisbon, Portugal
Address correspondence to:
Susana Amaral Pereira
Centro Hospitalar Universitário de Lisboa Central - Rua José António Serrano, 1150-199 Lisbon,
Portugal
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Article ID: 100098Z10SP2021
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Pereira SA, Fino R, Montalvão P, Doutel D, Pocinho R, Magalhães M. Malignant peripheral nerve sheath tumor of the infratemporal fossa: Case report and literature review. J Case Rep Images Oncology 2021;7:100098Z10SP2021.ABSTRACT
Introduction: Malignant peripheral nerve sheath tumor (MPNST) is rare soft tissue sarcoma that arises from a peripheral nerve or shows nerve sheath differentiation.
Case Report: We present a case of a 43-year-old female diagnosed with neurofibromatosis type 1 and with a plexiform neurofibroma of the infratemporal fossa (IF) evolving for about four years. Examination revealed facial asymmetry with the prominence of the right hemiface, without facial pain, motor deficits or facial numbness. The magnetic resonance imaging (MRI) showed a lesion in right IF with well-defined limits and 18F-FDG positron emission tomography/computed tomography (PET/CT) showed intense metabolism that was attributed to malignant transformation. Cytology confirmed a MPNST. Excision of the IF lesion by maxillary swing approach was performed. The anatomopathological evaluation of the lesion revealed a 7×6×3 cm high grade MPNST arising within a neurofibroma. The excision was juxtalesional. The tumor was staged at pT3cN0M0, IIIB. The patient underwent adjuvant radiotherapy. Currently, after 12 months of follow-up, the patient is asymptomatic, without neurologic deficits. The MRI performed three months after the end of radiotherapy shows no evidence of tumor recurrence.
Conclusion: The only known definitive curative treatment for MPNST is complete surgical resection with wide negative margins. The maxillary swing approach delivers wide tumor exposure, providing controlled dissection with minimizing morbidity. Despite maximal efforts, MPNSTs are usually highly aggressive. Increased tumor size and high-grade morphology, features which were observed in our patient, have been identified as factors that negatively affect long-term survival. We encourage a multidisciplinary approach in the management of these rare tumors to optimize outcomes.
Keywords: Head and neck neoplasms, Malignant peripheral nerve sheath tumors, Neurofibromatosis type 1
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Author Contributions
Susana Amaral Pereira - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Rui Fino - Conception of the work, Design of the work, Analysis of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Pedro Montalvão - Conception of the work, Design of the work, Analysis of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Delfim Doutel - Acquisition of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Rute Pocinho - Acquisition of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Miguel Magalhães - Conception of the work, Design of the work, Analysis of data, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2021 Susana Amaral Pereira et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
