下颌骨外周原始神经外胚层瘤病例报道及文献复习  

Peripheral primitive neuroectodermal tumor of the mandible: a case report and literature review

作　　者：卜海力齐古丽·麦麦提图尔荪 帕提姑丽·吾斯曼 阿地力·莫明[1] BU⁃HAILIQIGULI Maimaitituersun;PATIGULI Wusiman;ADILI Moming(Department of Oral and Maxillofacial Traumatol⁃ogy and Orthognathic Surgery,The First Affiliated Hospital of Xinjiang Medical University,Urumqi City 830054,China)

机构地区：[1]新疆医科大学第一附属医院口腔医学中心颌面创伤正颌外科

出　　处：《口腔疾病防治》2019年第12期784-787,共4页Journal of Prevention and Treatment for Stomatological Diseases

摘　　要：目的探讨下颌骨外周原始神经外胚层瘤(peripheral primitive neuroectodermal tumor,pPNET)的临床、影像学及病理特征,并进行相关文献复习提高对下颌骨pPNET的认识和诊治水平。方法对新疆医科大学第一附属医院诊治的1例下颌骨pPNET患者的临床及影像学特点、病理检查、治疗和预后进行观察,结合文献复习进行分析。结果该患者临床表现为下颌骨渐大性肿物,质地偏硬,边界不清,累及牙松动,伴下唇麻木;CT、MRI均表现为溶骨性及侵袭性的生长方式手术行下颌骨肿物切除及下颌骨部分截断性切除钛板重建术,术后病理切片HE染色镜下表现为小圆细胞肿瘤,免疫组化显示Vimentin及Fli⁃1表达为阳性,病理诊断为外周原始神经外胚层瘤。患者术后未行放化疗,随访9月后因肿瘤复发死亡。经相关文献复习,pPNET是一组罕见的高度恶性的小圆细胞肿瘤,多发生于儿童和青少年,恶性程度很高,病程短、转移快,主要的转移途径为血性转移,绝大多数患者在2年内死亡,3年生存率仅30%,5年生存率不超过10%。影像学上一般无特异性,故容易被误诊,最终的肿瘤类型依靠病理诊断。目前的治疗方法以手术完整切除为主,结合术后放化疗,但必要时,对于不同的患者给予个体化治疗很关键。结论pPNET恶性程度高、易复发转移、预后差,早期确诊及治疗极为重要。Objective To explore the clinical,imaging and pathological characteristics of mandibular peripheral primitive neuroectodermal tumors,and to review relevant literature to improve the understanding and diagnosis of pPNET in mandible.Methods The clinical and imaging features,pathological examination,treatment and prognosis of a case of mandibular pPNET diagnosed and treated at the First Affiliated Hospital of Xinjiang Medical University were observed,and analyzed a literature review.Results The patient′s clinical manifestations were an enlarged mass of the mandible,hard texture,unclear borders,involving loose teeth,and numbness of the lower lip;CT and MRI showed osteolytic and aggressive growth patterns.The mandibular tumor was resected and the mandibular partial trun⁃cated resection was performed on the titanium plate.Postoperative pathological sections showed small round cell tumors under HE staining and Vimentin and Fli⁃1 were positive,and the pathological diagnosis was pPNET.The patient did not undergo chemoradiotherapy after surgery and died of tumor recurrence after 9 months of follow⁃up.A review of the relevant literature revealed that pPNETs are a group of small round cell tumors,which are more common in children and adolescents.pPNETs have a high degree of malignancy,a short course of disease and fast metastasis.The main route of metastasis is through the blood circulation.Most patients die within 2 years,the 3⁃year survival rate is only 30%,and the 5⁃year survival rate is less than 10%.Imaging is generally nonspecific;therefore,pPNETs are easily mis⁃diagnosed.The final tumor type is determined by pathological HE staining and immunohistochemical characteristics.Current treatment methods are mainly complete surgical resection combined with postoperative radiotherapy and chemo⁃therapy,but it is critical to provide individualized treatment to patients when necessary.Conclusion pPNETs have a high degree of malignancy,easy recurrence and poor prognosis,so early diagnosis and treatment are extreme

关 键 词：外周原始神经外胚层肿瘤 Ewing′s肉瘤 下颌骨 溶骨性破坏 小圆细胞肿瘤 Vimentin蛋白 Fli-1蛋白 手术切除 肿瘤复发 预后 

分 类 号：R78[医药卫生—口腔医学] R739.8[医药卫生—临床医学]