Zhong Wangyan, Wang Cheng, Ye Wanli, Wu Dongping
Department of Radiation Oncology, Shaoxing People's Hospital, Shaoxing Hospital of Zhejiang University, Zhejiang, China.
Department of Pathology, Shaoxing People's Hospital, Shaoxing Hospital of Zhejiang University, Zhejiang, China.
Ear Nose Throat J. 2023 Jan;102(1):NP19-NP23. doi: 10.1177/0145561321989436. Epub 2021 Jan 21.
Olfactory neuroblastoma (ONB) is an ectodermal malignant tumor originating from the olfactory nerve epithelium of the nasal cavity and paranasal sinus. Olfactory neuroblastoma is a rare cancer, with an estimated incidence of 0.4 per million. Olfactory neuroblastoma often occurs in the top of the nasal cavity or near the lateral wall of the middle turbinate, which accounts for approximately 3% to 5% of the nasal cavity and paranasal sinus tumors. Olfactory neuroblastoma primarily in the nasopharynx is rare and tends to be misdiagnosed as nasopharyngeal carcinoma. Herein, we presented a case of ONB of the nasopharynx with recurrent nasopharyngeal tumor and by lymph node metastasis in both sides of the neck 14 months after surgical removal of the primary ONB. Long-term disease-free survival was achieved by definitive radiotherapy. Surgery combined with radiotherapy or definitive radiotherapy is recommended for primary ONB of the nasopharynx, radiotherapy target delineation including nasopharynx and pharyngeal lymph nodes, and neck lymph node drainage area of level Ib, Ⅱ, and Ⅲ.
嗅神经母细胞瘤（ONB）是一种起源于鼻腔和鼻窦嗅神经上皮的外胚层恶性肿瘤。嗅神经母细胞瘤是一种罕见的癌症，估计发病率为百万分之0.4。嗅神经母细胞瘤常发生于鼻腔顶部或中鼻甲侧壁附近，约占鼻腔和鼻窦肿瘤的3%至5%。原发于鼻咽部的嗅神经母细胞瘤较为罕见，易被误诊为鼻咽癌。在此，我们报告1例鼻咽部嗅神经母细胞瘤病例，该患者在手术切除原发嗅神经母细胞瘤14个月后出现鼻咽部肿瘤复发并伴有双侧颈部淋巴结转移。通过根治性放疗实现了长期无病生存。对于鼻咽部原发嗅神经母细胞瘤，推荐手术联合放疗或根治性放疗，放疗靶区勾画包括鼻咽部、咽淋巴环，以及Ib、Ⅱ和Ⅲ区颈部淋巴结引流区。
