鼻腔鼻窦肌上皮癌的生物学行为及诊治

The behaviour,diagnosis and treatment of sinonasal myoepithelial carcinoma.

目的 提高临床医生对鼻腔鼻窦肌上皮癌的认识。方法 回顾性分析我科近期收治的两例鼻腔鼻窦肌上皮癌临床资料 ,并复习文献。结果 1例为女性 ,4 7岁 ;1例为男性 ,5 4岁。临床表现为鼻塞、鼻腔暗红色肿物。鼻窦CT示鼻腔占位性病变。术前病理及免疫组化 (S - 10 0蛋白、平滑肌动蛋白、细胞角蛋白、波形蛋白等呈阳性染色 )确诊“肌上皮癌”。两例均行鼻侧切开鼻腔肿瘤切除术 ,例 1术后 5月出现颈淋巴结转移 ,术后 11月死于全身骨及肝转移。例 2术后 8月死于肺转移。结论 鼻腔鼻窦肌上皮癌极为罕见 ,迄今为止 ,仅 8例报道。综合文献 ,该瘤具有生长迅速、广泛侵犯周围组织、颈部淋巴结转移率高、血行转移率高、治疗后易复发、预后差等特点。诊断依赖病理及免疫组化。治疗以根治性手术为主 ,对于cN0的患者 。

Objective To enhance the recognition of sinonasal myoepithelial carcinoma.Methods Two patients with sinonasal myoepithelial carcinoma were diagnosed and treated recently,at the same time the literatures were reviewed.Results One patient was 47-year old female, the other was 54-year-old male.They presented with the snuffle symptom and a wine neoplasms in the nasal cavity. The paranasal sinus computerized tomography show a sign of a space-occupying lesion in the nasal cavity. In preoperation, histology supplemented by immunohistochemistry, confirmed the prescence of a myoepithelial carcinoma. A lateral rhinotomy was performed. Case one was found cervical lymph node metastasises 5 months after surgery, and died of the bone metastasis and liver metastasis 11 months after surgery. Case two died of lung metastasis 8 month after operation.Conclusion Sinonasal myoepithelial carcinoma is very rare tumor and,to date only 8 cases has been reported.According to the two cases and literatures, the characteristics of the tumor are rapidly enlarging,extensively invading the surrounding tissues,high rate of lymph node metastasis high rate of distant metastasis, very frequent recurrence after surgical excision, and poor prognosis. Diagnosis is depended on histology and immunohistochemistry. It must be treated radically. The elective neck dissection is generally necessary.