鼻腔、颞骨神经内分泌癌的生物学特征及诊治

Biological characteristics and treatment of nasal cavity,temporal bone neuroendocrine carcinoma

目的:报道2例原发于鼻腔鼻窦、颞骨小细胞神经内分泌癌,并探讨其临床生物学特征及诊治。方法:回顾性分析2例头颈神经内分泌癌的临床资料,并复习文献。结果:1例为女性,68岁,临床表现为进行性左鼻塞、鼻腔暗红色肿物。鼻窦CT示左鼻腔占位性病变,行手术加放化疗、靶向治疗,带瘤生存。1例为女性,70岁,临床表现为右耳流脓、耳痛、面瘫。先后行两次手术和两次放疗。第二次手术及放疗后1个月死于肺转移、全身多器官功能衰竭。结论:鼻腔鼻窦和颞骨神经内分泌癌极为罕见。该瘤具有临床表现不典型、生长迅速、侵犯广、转移率高、预后差、差异大等特点。诊断依靠病理及免疫组化。治疗应根据不同的临床分期和病理分型,实施以手术为主的综合治疗。

Objective：To report 2 cases of primary temporal bone small cell neuroendocrine carcinoma in the nasal cavity and paranasal sinuses, and study the biological characteristics and treatment. Methods：A retrospective analysis of two cases of neuroendocrine carcinoma of the head and neck clinical data was performed and review of the litera- tures. Results： Case 1 female, 68 - year - old, clinical manifestations were of progressive left nasal obstruction, dark red nasal tumor. The nasal CT showed left nasal cavity lesions, surgery plus chemotherapy, and targeted therapy were given, survive with tumor. 1 case of female, 70 - year - old, with right ear pus, earache, facial paralysis, treated with two operations and radiotherapy. After the second operation and radiotherapy and 1 month later, died of lung metasta- sis. Conclusion：The sinonasal and temporal bone neuroendocrine carcinoma is extremely rare. The tumor has no typi- cal clinical presentation, rapid growth, extensive violations, the prognosis is poor. Diagnosis relies on pathological and immunohistoehemical staining. Treatment should be based on different clinical stages and pathological type, compre- hensive and surgery could be chosen.