口腔婴儿黑色素神经外胚瘤的诊断及治疗

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.

Diagnosis and management of oral melanotic nenroectodermal tumor of infancy

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 intra-oral 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.