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A malignant peripheral nerve-sheath tumor: A CARE case report
1Service d'ORL, de Chirurgie Cervico-Faciale, Hôpital Henri Mondor, Assistance Publique des Hôpitaux de Paris, Univ Paris Est Créteil, INSERM, IMRB, CNRS EMR 7000, 94010 Créteil, France.
Malignant peripheral nerve-sheath tumor (MPNST) in the maxillary sinus is rare. Surgical resection and radiotherapy offer a favorable prognosis, with no recurrence observed in this case.
Area of Science:
- Oncology
- Surgical Pathology
Background:
- Malignant peripheral nerve-sheath tumor (MPNST) is an exceptionally rare sarcoma affecting the nasal cavity and paranasal sinuses.
- Its non-specific presentation and diagnostic challenges contribute to limited physician awareness.
Observation:
- A 62-year-old woman presented with progressive right cheek and hard palate swelling.
- Imaging revealed a maxillary sinus mass with significant osteolysis of surrounding structures.
- Histopathological analysis confirmed a low-grade MPNST.
Findings:
- Complete surgical resection of the maxillary sinus MPNST was achieved.
- Adjuvant radiotherapy (60Gy) was administered post-operatively.
- A 2-year follow-up demonstrated no evidence of tumor recurrence.
Implications:
- MPNST of the sinonasal region requires prompt diagnosis and aggressive management.
- Wide surgical resection is crucial due to the high risk of recurrence.
- Adjuvant radiotherapy should be considered, especially when complete resection is challenging due to anatomical proximity to critical structures.
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