Myxofibrosarcoma of the Maxillary Sinus

Background Myxofibrosarcoma (MFS) is a common sarcoma in the extremities of older individuals but is extremely uncommon in the head and neck region. Diagnosis may be challenging but is critical to the management of the patient. We discuss the radiographic and histopathologic characteristics of this...

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Main Authors: Anni Wong B.A.,M.S., Richard Chan Woo Park M.D.,F.A.C.S., Neena M. Mirani M.D., Jean Anderson Eloy M.D.,F.A.C.S.
Format: Article
Language:English
Published: SAGE Publishing 2017-06-01
Series:Allergy & Rhinology
Online Access:https://doi.org/10.2500/ar.2017.8.0200
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author Anni Wong B.A.,M.S.
Richard Chan Woo Park M.D.,F.A.C.S.
Neena M. Mirani M.D.
Jean Anderson Eloy M.D.,F.A.C.S.
author_facet Anni Wong B.A.,M.S.
Richard Chan Woo Park M.D.,F.A.C.S.
Neena M. Mirani M.D.
Jean Anderson Eloy M.D.,F.A.C.S.
author_sort Anni Wong B.A.,M.S.
collection DOAJ
description Background Myxofibrosarcoma (MFS) is a common sarcoma in the extremities of older individuals but is extremely uncommon in the head and neck region. Diagnosis may be challenging but is critical to the management of the patient. We discuss the radiographic and histopathologic characteristics of this destructive tumor. The distinguishing features of MFS and its differential diagnosis are reviewed to familiarize the managing otolaryngologist with this rare entity. Methods A 61-year-old woman presents with a 6-week history of severe left facial pain and left eye pain. Imaging demonstrates significant right and left-sided maxillary sinus opacification with destruction of the left maxillary sinus as well as the left medial and inferior orbital walls. Results Histopathologic examination revealed spindle and stellate tumor cells of variable cellularity in myxoid stroma with cellular pleomorphism consistent with MFS of intermediate-to high grade. The patient underwent resection of the left-sided lesion with orbital exenteration and repair of the defect with microvascular free flap followed by postoperative radiotherapy. Conclusion MFS must be differentiated from other lesions with myxoid qualities. Histopathologic examination is required for definitive diagnosis. Management includes complete tumor excision with adequate tumor margins. Adjuvant postoperative radiotherapy should be considered for larger tumors with positive resection margins or lesions of intermediate-to-high grade.
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spelling doaj.art-61df20fa46c04bd496116017c731d2ec2022-12-21T19:03:29ZengSAGE PublishingAllergy & Rhinology2152-65672017-06-01810.2500/ar.2017.8.0200Myxofibrosarcoma of the Maxillary SinusAnni Wong B.A.,M.S.0Richard Chan Woo Park M.D.,F.A.C.S.1Neena M. Mirani M.D.2Jean Anderson Eloy M.D.,F.A.C.S.3 Department of Otolaryngology—Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey Department of Otolaryngology—Head and Neck Surgery, Rutgers New Jersey Medical School, Newark, New Jersey Department of Ophthalmology and Visual Science, Rutgers New Jersey Medical School, Newark, New Jersey Department of Neurological Surgery, Rutgers New Jersey Medical School, Newark, New JerseyBackground Myxofibrosarcoma (MFS) is a common sarcoma in the extremities of older individuals but is extremely uncommon in the head and neck region. Diagnosis may be challenging but is critical to the management of the patient. We discuss the radiographic and histopathologic characteristics of this destructive tumor. The distinguishing features of MFS and its differential diagnosis are reviewed to familiarize the managing otolaryngologist with this rare entity. Methods A 61-year-old woman presents with a 6-week history of severe left facial pain and left eye pain. Imaging demonstrates significant right and left-sided maxillary sinus opacification with destruction of the left maxillary sinus as well as the left medial and inferior orbital walls. Results Histopathologic examination revealed spindle and stellate tumor cells of variable cellularity in myxoid stroma with cellular pleomorphism consistent with MFS of intermediate-to high grade. The patient underwent resection of the left-sided lesion with orbital exenteration and repair of the defect with microvascular free flap followed by postoperative radiotherapy. Conclusion MFS must be differentiated from other lesions with myxoid qualities. Histopathologic examination is required for definitive diagnosis. Management includes complete tumor excision with adequate tumor margins. Adjuvant postoperative radiotherapy should be considered for larger tumors with positive resection margins or lesions of intermediate-to-high grade.https://doi.org/10.2500/ar.2017.8.0200
spellingShingle Anni Wong B.A.,M.S.
Richard Chan Woo Park M.D.,F.A.C.S.
Neena M. Mirani M.D.
Jean Anderson Eloy M.D.,F.A.C.S.
Myxofibrosarcoma of the Maxillary Sinus
Allergy & Rhinology
title Myxofibrosarcoma of the Maxillary Sinus
title_full Myxofibrosarcoma of the Maxillary Sinus
title_fullStr Myxofibrosarcoma of the Maxillary Sinus
title_full_unstemmed Myxofibrosarcoma of the Maxillary Sinus
title_short Myxofibrosarcoma of the Maxillary Sinus
title_sort myxofibrosarcoma of the maxillary sinus
url https://doi.org/10.2500/ar.2017.8.0200
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