颌面部神经内分泌癌13例临床分析

Clinical analysis of 13 cases of maxillofacial neuroendocrine carcinoma

目的探讨颌面部神经内分泌癌(NEC)的临床特点与预后。方法回顾性分析解放军总医院1997年1月至2016年1月收治的13例临床病理检查明确诊断的颌面部NEC患者临床信息,对其一般资料、治疗方式、病理检查和临床预后进行分析。结果 13例患者中男4例(30.8%),女9例(69.2%);平均年龄(60.4±4.8)岁。临床过程呈侵袭性,其中原发占位未转移2例(15.4%),临近器官及区域淋巴结转移9例(69.2%),远处器官转移(转移至卵巢、肺)2例(15.4%)。肿瘤累及腮腺5例、上下颌3例,累及舌部、面部、腭部、口底颌下及颈部各1例。苏木精-伊红(HE)染色均表现为小细胞NEC。肿瘤免疫组化分析发现,嗜铬素(Cg A)染色阳性7例(53.8%)、突触素(s Syn)染色阳性11例(84.6%),神经元特异性烯醇化酶(NSE)染色阳性10例(76.9%)。Ki-67增殖指数≥50%者占69.2%,Ki-67增殖指数<50%者占30.8%。13例患者均进行了手术治疗,除2例拒绝放化疗外,根据患者病情都进行了辅助放疗和化疗。术后又复发者有5例,内脏器官转移者2例,至随访结束,目前存活者有2例。结论颌面部NEC的临床表现无特异性,容易出现淋巴转移及临近器官转移。手术切除是目前最主要的治疗方法,辅助化疗可能使患者获益,但总体预后较差。

Objective To investigate the clinical characteristics and prognosis of maxillofacial neuroendocrine carcinoma（NEC）. Methods A retrospective study was performed,including 13 patients with maxillofacial neuroendocrine carcinoma which was diagnosed by clinical pathology in General Hospital of PLA between January 1997 and January 2016.The general clinical information,treatment,pathological examination and clinical prognosis were analyzed. Results Among the 13 patients,4 were male（30.8%）and 9 were female（69.2%）witha mean age of 60.4±4.8 years old. The course of the disease was aggressive. Two cases（15.4%）were primary cancer without metastasis;9 cases（69.2%）metastasized to adjacent organ and regional lymph node;2 cases（15.4%）metastasized to distant organ（ovary and lung）. Maxillofacial neuroendocrine carcinoma location in 13 cases was：5 in parotid gland,3 in upper/lower jaw,1 in tongue,1 in face,1 in submandibular area and 1 in neck. The hematoxylin-eosin（HE）pathological staining showed the histological type was small cell type. Tumor immunohistochemical analysis found that 7 cases（53.8%）were chromogranin A（Cg A）positive,11 cases（84.6%）were synapsin（Syn）positive and 10 cases（76.9%）were neuron-specific enolase（NSE）positive. The cases with Ki-67 proliferation index≥50% accounted for 69.2%,and Ki-67 proliferation index50% 30.8%.The 5-year survival rate was 15.4%. Thirteen patients were undergone surgery. Adjuvant radiotherapy and chemotherapy were performed according to the patient＇s condition except two patients who refused adjuvant treatment. 5 cases suffered tumor recurrence and 2 cases had internal organ metastasis. Only 2 cases survived at the end of follow-up investigation.Conclusion Maxillofacial NEC shows no specific clinical manifestation. NEC is prone to develop lymph node metastasis and adjacent organs metastasis. Surgical operation is the primary treatment so far. Postoperative adjuvant radiotherapy and chemotherapy may be beneficial to patients,but the overall prognosis is poor.