中文摘要：

目的：探讨口腔婴儿黑色素神经外胚瘤的诊断要点及治疗原则。方法：对北京大学口腔医学院1980年至2007年8月间收治的13例口腔婴儿黑色素神经外胚瘤病例进行回顾性研究，复习临床资料及病理切片。结果：9例口腔婴儿黑色素神经外胚瘤发生于上颌骨，3例位于下颌骨，1例位于颊部。发病年龄从2个月至7个月。临床症状均为肿物，5例术前检查发现肿物为紫红色或蓝黑色。肿物生长迅速，3例术前诊断为恶性肿瘤，仅有1例术前临床诊断为婴儿黑色素神经外胚瘤。9例有随访结果，2例确定复发，复发时间均为术后1个月，1例死亡。1例切除不完全的病例术后19年无复发。结论：口腔婴儿黑色素神经外胚瘤具有典型的发病部位与发病年龄，临床检查时应注意肿物的颜色。肿瘤常生长迅速，具有局部侵袭性。对患者术后半年内要密切随访；病理诊断要与儿童小圆细胞恶性肿瘤相鉴别；相对保守的手术为首选治疗方式。

英文摘要：

Objective： To investigate the clinicopathological features of oral melanotic neuroectodermal tumor of infancy （MNTI）. Methods： We retrospectively reviewed the clinical and pathological data of 13 cases of oral MNTI treated in Peking University School and Hospital of Stomatology from 1980 to 2007. Results： The age of patients ranged from 2 to 7 months old. Nine lesions occurred in the maxilla, 3 in the mandible and 1 in the cheek. The most common symptom of the disease was swelling. Five tumors were found purple-reddish or blue-blackish. The neoplasm grew rapidly. Only 1 case was diagnosed as MNTI and 3 cases were diagnosed as malignant tumor before biopsy. Two of 9 cases with follow-up information recurred in one month after surgery and 1 case dead of the tumor. One case with remnants of tumor did not recur after 19 years postoperatively. Conclusion： Oral MNTIs appear to occur in certain locations and people of certain age. Special attention should be paid to the color of the tumor during intraoral examination. This tumor grew rapidly and invasively and the patients require close follow-up within the first 6 months postoperatively. The pathologic differential diagnosis of MNTI should include other pe- diatric ＂small round cell＂ neoplasms. Conservative excision is the preferred treatment choice.