目的：探讨鼻腔鼻窦罕见肿瘤的影像学特征，提高对此类疾病的认识，从而辅助临床诊断和治疗。方法：回顾性分析79例经病理证实的罕见鼻腔鼻窦肿瘤的CT、MRI表现，总结其影像学特点。结果：79例中，神经内分泌癌16例，多数表现为扩张性、浸润性骨破坏，无骨质增生和硬化。蝶窦呈“鸽子”状。 28例恶性黑色素瘤MRI信号多样，典型信号少见，但混合信号较多见。 12例横纹肌肉瘤MRI强化多呈“葡萄样”强化及部分环状强化；嗅神经母细胞瘤10例，病灶与嗅粘膜分布区一致，多呈分叶状、边缘结节、“花环”强化，2例跨越颅内外生长，多发囊性病灶，周围有片状水肿带。 5例孤立性纤维瘤中，良性肿瘤形态规则，密度均匀，而恶性肿瘤形态不规则，密度不均，强化明显不均匀，呈“图案”状改变。肉瘤样癌2例，均呈分叶状，密度不均，板层状低密度影，有溶骨性骨破坏。 4例神经鞘瘤强化呈明显不均匀强化。 1例呈囊性坏死，1例呈钙化，周围结构受压，无损伤。神经纤维瘤1例，囊性成分较多，低信号分离，呈区室化强化。 1例副神经节瘤表现为动脉期中度强化，静脉期进行性强化，并伴有明显的肿胀骨质破坏。结论：鼻腔鼻窦罕见肿瘤具有鲜明的影像学特征。 CT和MRI能有效显示病变范围及邻近组织器官浸润程度，有助于临床早期诊断和分期。但确诊仍需依靠病理学和免疫组化。版权所有©《临床耳鼻咽喉头颈外科杂志》编辑部。

Objective:To explore the imaging features of rare tumors of nasal cavity and sinuses, and to improve the understanding of these diseases, thereby aiding clinical diagnosis and treatment. Methods:The CT and MRI findings of 79 cases of rare neoplasm of nasal cavity and sinuses confirmed by pathology were retrospectively analyzed, and the imaging features were summarized. Results:Among the 79 cases, there were 16 cases of neuroendocrine carcinoma, most showing expansive and infiltrative bone destruction without hyperosteogeny and sclerosis. The sphenoid sinus exhibited a "pigeon" shape. In 28 cases of malignant melanoma, MRI signals were diverse, typical signals were rare, but mixed signals were more common. In 12 cases of rhabdomyosarcoma, MRI enhancement mostly showed "grape-like" enhancement and partial ring enhancement; There were 10 cases of olfactory neuroblastoma, the lesions were consistent with the distribution area of olfactory mucosa, most of them were lobulated, marginal nodules, and "flower ring" enhancement, and 2 cases grew across intracranial and external, with multiple cystic lesions and surrounding flaky edema bands. In 5 cases of solitary fibrous tumor, Benign tumors had regular shape and uniform density, while malignant tumors had irregular shape and uneven density, The enhancement was obviously uneven and showed a "pattern" change. There were 2 cases of sarcomatoid carcinoma, both with lobed appearance, uneven density, lamellar low-density shadow, and osteolytic bone destruction. In 4 cases of schwannoma, the enhancement showed obvious inhomogeneous enhancement. One case showed cystic necrosis, one case showed calcification, and the surrounding structure was compressed without damage. There was 1 case of neurofibroma, with many cystic components, low signal separation and compartmentalized enhancement. One case of paraganglioma showed moderate enhancement in the arterial phase and progressive enhancement in the venous phase, accompanied by significant swelling bone destruction. Conclusion:Rare tumors of nasal cavity and paranasal sinuses have distinctive imaging features. CT and MRI can effectively show the extent of the lesions and the degree of infiltration into adjacent tissues and organs, which is helpful for early clinical diagnosis and staging. However, definitive diagnosis still depends on pathology and immunohistochemistry.Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.