Service 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.
Service d'Anatomo-Cyto-Pathologie, Centre Hospitalier Intercommunal de Créteil, 94000 Créteil, France.
Eur Ann Otorhinolaryngol Head Neck Dis. 2023 Nov;140(6):309-311. doi: 10.1016/j.anorl.2023.10.005. Epub 2023 Oct 19.
Malignant peripheral nerve-sheath tumor (MPNST) is an extremely rare sarcoma of the nasal cavity and paranasal sinuses. Non-specific clinical and radiological presentation and challenging histological diagnosis make it little known by physicians. We describe a case of maxillary sinus MPNST, following CARE guidelines.
A 62-year-old woman consulted for swelling of the right cheek and hard palate with several months' progression. CT and MRI revealed a tissue mass in the right maxillary sinus with osteolysis of the orbital and maxillary floors, hard palate and lateral and medial walls of the maxillary sinus. Biopsy confirmed diagnosis of low-grade MPNST. After total resection and 60Gy adjuvant radiotherapy, 2-year follow-up showed no signs of recurrence.
MPNST in the nasal cavity and paranasal sinuses is very rare. Because of a high risk of recurrence, wide resection should be implemented, possibly completed by radiotherapy if resection cannot be complete because of proximity to at-risk structures.
恶性外周神经鞘瘤（MPNST）是一种极为罕见的鼻腔和鼻窦肉瘤。非特异性的临床和影像学表现以及具有挑战性的组织学诊断使得医生对此病了解甚少。我们按照 CARE 指南描述了一例上颌窦 MPNST 病例。
一名 62 岁女性因右侧脸颊和硬腭肿胀数月就诊。CT 和 MRI 显示右侧上颌窦内有组织肿块，伴有眶底和上颌骨底、硬腭以及上颌窦外侧壁和内侧壁的骨溶解。活检证实为低级别 MPNST。行全切除和 60Gy 辅助放疗后，2 年随访未见复发迹象。
鼻腔和鼻窦的 MPNST 非常罕见。由于复发风险高，应进行广泛切除，如果由于临近高危结构而无法完全切除，则可能需要辅以放疗。
