涎腺恶性肌上皮瘤16例临床分析

Malignant myoepithelioma of the salivary glands: a report of 16 cases

目的:探讨涎腺恶性肌上皮瘤生物学行为及其有效治疗方法。方法:对1990年以来中山大学肿瘤医院收治的16例恶性肌上皮瘤患者进行临床病理分析,随访时间为2个月～12年。结果:16例患者中,男10例,女6例。年龄15～73岁,中位年龄40岁。发病部位以腮腺多发。全部病例均以肿块为首发症状。9例(56.3%)有颈淋巴结转移。病理以梭形细胞为主的8例,上皮细胞为主、透明细胞为主和混合型(含有梭形、上皮和透明细胞)的为少数。CT表现为局部有不规则的软组织团块影,密度不均匀,边缘不规则,肿块内可见低密度坏死区,与周围结构分界不清。免疫组化检查对S100、cytokeratin、vimentin和HHF35等阳性反应程度高。治疗采用以手术为主的综合治疗。总的3年生存率为90.9%(10/11),5年生存率为87.5%(7/8)。化疗有良好的控制率(71.4%)。结论:涎腺恶性肌上皮瘤是一种预后较好的恶性肿瘤,但局部复发率高,早期淋巴结转移率低,晚期淋巴结及远处转移率明显升高。早期患者手术可达到根治效果,晚期需行以手术为主的综合治疗,无手术指征者,姑息化疗(CTX+DDP+ADM/DTIC)有良好的近期缓解率。

OBJECTIVE： To investigate the clinicobiological behavior and effective treatment for malignant myoepithelioma from the salivary glands. METHODS： Clinicopathological analysis was done in 16 patients with malignant myoepithelioma from salivary glands hospitalized in our hospital since 1990. Follow-up time ranged from 2 months to 12 years. RESULTS：Sixleen patients included 10 men and 6 women, with age ranged from 15 to 73 years （median 40. 5 years）. The most of mnlignant myoepithelioma occurred in salivary gland and all presented firstly with mass. Nine （56.3%） patients had neck nodes metastasis. Pathologically, spindle cells mainly presented in eight patients, the rest few patients presented with cutaneous cells, opaque cells and mixed type （including spindle cells, cutaneous cells and opaque cells）. CT image showed irregular soft tissue mass with different density and irregular margin. Necrosis was seen in the middle of the mass without clear margin to surround structures. Immuniohistochemistry found high response in S-100, CK, Vimentin and HHF-35. All of the patients underwent multimodality treatmenl with surgery as the main. Overall 3-year and 5-year survival rate were 90.9 % and 87.5 % respectively. Chemotherapy had satisfactory effective rate （71.4%）. CONCLUSIONS： Malignant myoepithelioma in salivary glands has satisfactory prognosis. However, it has high rate of local recurrence. In early stage local metastasis is low, however, in advanced stage the local and distant metastasis are both significantly high. Early patients can be cured by surgery, but multimodality treatment including surgery is necessary for advanced patients. For unresectable patients, palliative chemotherapy （CTX ＋ DDP ＋ ADM/DTIC） has satisfactory response.